1967 SEMINAR==~~ · 2015. 10. 3. · ~===1967 seminar==~~ houston society of cunigal pathologists . pediatrics, pathological and radiological seminar james b. arey, m.d. professor

~===1967 SEMINAR==~~

HOUSTON SOCIETY OF CUNIGAL PATHOLOGISTS

. PEDIATRICS, PATHOLOGICAL AND RADIOLOGICAL SEMINAR

JAMES B. AREY, M.D. PROFESSOR OF PATHOLOGY

TEMPLE UNIVERSITY SCHOOL OF 'MEDICINE

PATHOLOGIST

ST. CHRISTOPHER'S HOSPrrAL FOR. CHILDRii_N

PHILADELPHIA,, P.ENNSYLVANIA

PRESEN1ED BY

JOHN A. KIRKPATRICK, JR., M.D. PROFESSOR. QF RADIOLOGY

TEMPLE UNIVERSITY 'SCHOOl OF- MEDICINE

RADIOLOGIST

ST. CHRISTOPHER'S H'OSPITAL FOR CHILDREN

PHilADELPHIA, PENNSYLVANIA

HOUSTQN, TEXAS

APRIL 22, 19G7

HOUSTON SOCIETY OF CLD!ICAL PATHOLOOISTS

1967 SE!ID!AR

"PEDIATRICS , PATHOLOOICAL AND RADIOLOOICAL SEMINAR"

DIAGNOSES

CASE NO.

1. Cytomegal.ic inclusion disease of tvv.y 2 . Alveolar proteinosis lv~

3·

4.

i t ll i •~ ..... ~' G an ce pneumon a

Cystic adenomatoid I• - I f • ~ ~ df. l ""0'

5. Atresia of pulmonary vein I lv"1 )

6. Ectodermal dysplasia

1·

a. 9.

10.

ll.

12.

13.

14.

Juvenile fibr omat osis J ~yJ n~·....,p_ 14esenchymoma , ~....,(,.J. , oC r.~ l:.

Melanotic progonoma ~f ~tc»J.l Juvenile nas opharyngeal angiofibroma

Renal vein thrombosis

Takayasu's disease

Rhabdomyosarcoma 1 .... ,\ -1, , ..Q. o~ 1-LW 'l , IH

Pre-sacral teratoma, recurrent , ""'"\. \~ •

15. Wolman 'a disease i"' """Ml c., .JL

16. Cervical neuroblastoma

17. Wilms' with calcification

18. Psoas Abscess

19. Focal nodular hyperplasia 0 f" \ I 11'.1/

20. Eosinophilic granuloma of """ ,.

•

St. Luke's Episcopal Hospit a l Texas Medical Center H.ouaton, Texas

A:pr il 22, 1967

HOUSTON SOClETY OF CIJ:NICAL PATHOIDGISTS 7TH ANNUAL_ SE11INA,R "lEDIATBICS, PA!rHOWGICAL AND RAJ)IOLOGI:CAL SEM!iNAR"

Sat~, April 22, 1967

CASE 1. Pytomega.lic inclusion disease

1. Smith, M.G. and Vellios, F.: Inclusion disease · or generalized salivary gland virus infection, A.M. A. Arch. Path, 50: 862-6841 1950.

2. Gallagher, H.S ,: Cytomegalic inclusion disease of infancy. Report of a case associated With cysts of the lung; With recovery followi'!1g lobectom,y, Am,. J. Olin. Path, 22:1147-1152, 1952.

3. McElfresh, A.E, and Arey, J ,B,: Generalized cytomegalic inclusion disease; J. Pediat. 51:146-156, 1957 •

4• Nelson, J .S. and Wyatt, J . P.: Salivary gland virus disease, Medicine 38:223- 2411 1959·

5· Vanier, T.M.:. A case of cytomegalic inclusion dis·ease, Thorax 15: 259- 261, 1960,

6. Symners, w. St, C.: Generalized cytomegalic inolusion-boey disease associated With pneumocyatis pneumonia in adults, A report of three cases with Wegene~•s granulomatosis, tbrombic purpura, and Hodgkin' s disease· as predisposing conditions, J, Ol in. > Path, 13:1-211 1960,

7• \~elJ,er1 T. H., Hanshaw, J .B.. and Scott, D'I1. E.: Virologic and clinical observations on cytomegalic inclusion disease, New Eng. J . l•fed, 266:1233- 1244, 1962.

a. Zue1zer1 W.W. and Stu1berg, C,S., Fags, R,H., Terqya, J. and Brol.l{';h, A,J, : Etiology and :pathogenesis of acquired hemolytic anemia, Transfusion 6:438-461, 1966.

9. Gaj1-Peczalska.1 K.: Cytomegalic inclusion disease, Arch. Dis. Childh, 42:14-19, 1967.

10, Rinker , C.T. and McGraw, J,P,: Cytom:ega.lic inclusion disease in childhood leuke.mia, Cancer 20:36-391 1967 • .

CASE 2. Puimerr alveoler proteinosis

1. Eosen,_ S,H., Castleman, B. and Liebow, AoAol Pulmonary alveolar proteinosis, New Eng. J. l

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} . Hall, G, F. M. s Pulmonary alveolar proteinosia, Is.ncet l1 1}8}- 1}85, 1960.

4• Medl ey, D, R. K. and Brown, R,: Pulmonary alveolar proteinosis, Is.ncet 111}85- 1388, 1960.

5· De Sanotis, P. N. : Pulmonary alveolar proteinosis. A reView of the findinge and theories to da.te , with a digression on pneumocystis oarinii pneumonia, Boston M, Quarterly l}s 19- 35, 1962.

6 , Andriola , V. T. , Ballas, 11, and Wilson, G. 1 .: The association of nocardiosis and pulmonary alveolar Prot einosie. A oase s t udy, Ann. Int . Med. 601266- 275, 1964.

7• Carleen, E,T., Hill, Jr. , R.B. and Rowlands , Jr., ·D. T.s Nocardiosis and pulmonary alveolar proteinosis, Ann. Int. Med. 60:275-281, 1964·

CASfJ } . Measles giant cell pneumon.ia,.

1, Cor bett , E,V,: The Vixceral lesions in·measl es . With a report of Xoplik spots in the colon, Am. J, &th. 21: 905- 919. 1945·

2 , Tompkins, V. and Maoaulay, J.C.s A obaraoteristio cell in resa1 secretions during prodromal measles, JAHA 1571711, 1955·

3 • Enders, J , F. , McCarthy, K., Mitus, A. and Chea:tham, W. J . s Isolation of measles virus at autops,y in oases of giant- cell pneumonia without rash, New Eng, j , Med. 2611875- 881, 1959.

4 • .Mitus , A., Enders, J , F,, Craig, J .H. and Holloway, A.s Fersistenoe of measles virus and depression of antib~ formation in patients with giant-~ll pneumonia after measles , New Eng. J . Med, 2611882-889, 1959.

CASE 4• Cvstic ad,enomatoid wlforma,tion •

1. C'n'in, K.Y. and Tang, H. Y.: Congenital adenoma:t oid mal-f ormation of one lobe of a lung with general anaaaroa, Arch. Eath. 481221- 229, 1949·

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2. Craig, J . l-1., Kirlqatrick, J . and Neuhauser, E.J! . D.: Col181lnital cystic adenomatoid malformation of the lung in infants, Am. J . Roentgenol . 76:516-526, 1956.

3· Goo~ar, J.E . and Shilltoe, A. J .: Adenomatoid hamartoma of the lung in a newborn infant , J . Clin. Bath. 12:172-174, 1959·

4• llain, G. O.: Congenital adenomatoid malformation of the lung, Dis. Chest 36:430-433, 1959.

CASE 5. Atresia of the common pplmonary vein.

1. Lucas, R.v • .Jr~, Woolfroy, B. F .. , Anderson, R.c., Lester, R. G. and Edwards, J.E.: Atresia of' the common pulmonary vein, Bediatrica 29:729-739,1962.

2. Lucas, R.V. Jr., Anderson, R. c. , Amplatz , K. , Adams, P. Jr. and Edwards, J .E ol Col181lni tal causes of pulmonary venous obstruction, Ped. Clin. N. Amer. 10:781-836, August , 1963.

3• Carter, R.w. and Vaughn, H.l•!.: Ool181lnital pulmonary ~pbangieotaais. Report of a case with roentgen findings , Am. J. Roentgenol . 86:576-578, 1961.

Javett , S . N., Webster , I . and Braudom J . L. : Congenital dilatation of the pulmonary ~pbatias , PediS.trios 31:416-425. 196;.

5· Ekelund, H., - Bl.lmSti~,s . and Ostberg, G.: Oongenoital pulmonary lympbaniectasis, Aota Paediat . Soandinavica 55:121-125, 1966.

CASE 6. CoMeni tal a.nhid,rotic ectodermal dysp]l!,sia. .

1. Clou.eton, H.R.: The major £oms of hereditary ectodennal d;yspla.sia (with a.n qutopsy a.Xld biopsies on the anhidrotic type), Canad. 11.A.J . 40: 1-7, 1939·

2 . Lipton, I ., and Roberta , M.H. : Heriditary ectodennal d;yaplaaia of the anhidrotic type, Am. J . Dis. Child. 70: 504-509 , 1950·

3• DeJager, H.: Congenital anhidrotic ectodermal dJ'Splaaia: Ce.se report , J . lath. & llact. 90:321-322, 1965.

-4-

CAlml 7. Juveliile fibroma, tosis.

1. Stout, A.P. : Juvenile fibromatosis, Cancer 7:953- 978,1954•

2. Stout, A.P.: Fibrosarcana in infants a.nd children, C.3.ncer 15:1028-1040, 1962.

3• Bartlett, R.c., Qtis, R.D. and Ie.a:ks.o, A.O,: l

~~--~--------~--------------------,

- 5-

~ . :Blanc, W,A., Rosenblatt , P, and Wolff, J ,A,: Melanotic progonoma ("retinal anlage" tumor) of the shoulder in an infant. A case report , Cancer 11&959-963 1 1958.

4• Wrie , B:. I .: Congenital melanoca.rcill0111111 melanotic adamantinoma, retinal anlage tumor, progonoma, and pig111ented epulis of in-fancy. Summary and reView of the literature and report of the first case in an adult , Cancer 14&1090-1108, 1961.

5. Porter, J .E. and C=inge, G,O, Jr ,& !'felanotic progonoma of the maxilla. Case report of so- called retinal anlage tumor, Arch E&th. 76:267-270, 196~ .

6, Reyes , B:, A, 1 Gonzales- .Angulo, A, and Nava.rete-Reyna, A, I "Retinal anlage tumor of infancy'' 1 Report of a case occurring in ·the anterior fontanel, J , Pediat, 64• 268-2711 1964,

1· f

-6-

6, Balogh, K. Jr, and Caulfield, J .ll,: Ultrastructure and hietoohemietry of juvenile nasopharyngeal angiofibromas, Proc, Am, Assoc. Path, & :Bact ,, l1a.:rch 10, 1967 (ab-stract) ,

7• Svoboda, D. J . and Kirchner, F , : Ultrastructure of naso-~;ngeal angiofibromas, Cancer 19: 1949-1962, 1966.

CASB 11. Renal vein tbroJ!!bosis , old,

1, Pollak, V.E ,, Ea.rk, R, H,, l'irani , C, L,, Shafter , H. A. l·fu.ehrcke, R,C, : Renal vein thrombosis and the nephrotic syndrome, Am. J , Had, 21::496-520, 1956,

2. Kaufma.Dn, H. J .: Renal vein thrombosis . 1. ~ incidence in infancy and childhood; 2. Sex incidence+~. Incidence of unilateral and bilateral involvment, A.M.A. Am. J , Dis. Child, 95:;77-;84. 1958.

; , Hiller, H. C. and :Benjamin, J . A.: Acute idio:pa,thic renal vein thrombosis in infants , Pediatrics ;0:247-252, 1962.

4• Morris , J ,F,, Ginn, H.E . and Thompson, D.,D.: Unilateral renal vein thrombosis associated with the nephrotic syndrome, Am;- J . Jofed. ;4:867-874, 196;~

5· Stark, H,: Renal vein thrOmbosis in infancy. l!ecovery with-out neJin'ectom;y, Am. J , Dis. Child, 108:4;0-4;5, 1964.

P:rimery: =rti"~is (pfsdese disea,se: e,ortic arch syndrome: T su' s dise se),

l: , Gelfand, I•r.: Giant cell ateritia with aneurysmal formation in an infant, :Brit . Heart J , 17: 264- 266, 1955.

2 , Dana.:raj , T , J , and Ong, H.W.: Primary arteritis of abdominal aorta in children causing bilateral stenosis of renal arteries and hypertension, Circulation 20:856-86;, 1959.

; . Iaaacson, c,: An idio:pa,thic aortitis in young Africans, J , Path, & :Bact, 81:69- 79, 1961,

4• Okuni, H,, Iijima, ~r. KDba;y'a.shi , N. and Saito>Y: Primary arteritis in children report of three cases, Paediatria Universitatis To~c 7:;2- ;5, 1962.

5, Waeenv.oort , O,A, , Harris , L.E, :Brown, A, L, Jr , and Veeneklaas, G.M. H,: Giant- cell arteritis with aneurysm formation in children, Pediatrics ;2:861-867, 196;.

-1-

6, Heggtveit1 H,A,, He:nnip;a.r1 G,R. and l1ort'ione1 ' ToGo: Fanaortitis, Am, J , Fath. 421151- 172, 196~.

7• Lee , x;,s , and R:!.nvik1 R.: Primary arteritis wi th hypertension in ohildren - A report of f our. cases wi th primary arteritis causing a ty-pical C!J:&rca tion of . .the abdominal aorta and hypertension, J , Korean M.A. : 712al-2911 1964.

a , Warshaw, J.B, and SJ8.0h1 M, S ,;Tak:!cye.au1s disease (primary aortitis) in ohildhood, Case report with reyiew of l i terature, Pediatrics ~5:620-626 1 1965,

9, Zerpe. , F,, Hirscbhl.l.ut1 E., Ferrer, A,, Oapr:i.ks1 M,A, and Dubois, E,1 T~su' s disease (primary aortitis)! re

-8-

CASE 14 Malignant sacrococcygeal teratoma, (emb:cyopal oa,rcinoms,).

1. RhOden , A.D.: Precocious sexual and somatic developejllent in a male infant with a. presacral teratOma. containing aud.rogen-pr_oducing tissue'. 1vi th a discussion of the mechanism · of -o:f precocity qa.used by t ·eratomas1 J. Clin. Endoorinol. 4: 185-19:?, 1944·

2. Gross , R.E., Cla.twortby, H.w. Jr.- and Meeker, I .A. Jr.: Sacrococcygeal t eratomas i .n infanta and children. A report of 40 oases, Surg., G,ynec. & Obst. 92: 341-3541 1951.

3. l1ostofi; F. K.: .Infantile testicular tumors , Bull. New York Aca.d. Med. 28: 684- 687, 1952.

4• Rusche, C.: T1~elve cases o:f teet ic.Ular tumors ··occurring during infancy and childhood, J . Pediat. 40: 1920199, 1952.

5· Hickey, R.c. and !cyton, J.M.: Saorococcygea.1 teratoma.. · Emphasis- on the biol ogical histor-y and earl;y therapy, Cancer 7:1031- 10431 1954•

6. Gwinn, J . L., Dockerty, M.B. and Kennecy, R. Ii. J .: Presa;cral t eratomas in infancy and childhood, Ped.:i!l.trics 16: 239- 249 1 1955·.

7 • Magner, D., Campbell , J .s. and \Viglesworth, F. W .: Testicular adenocarcinoma 1nth clear cells, occurring i n infancY, Cancer 9: 165- 175, 1956.

8. Teoh, T. B·., Steward, J . K. and Willis, R.k.: The d:istinotive adenocarcinoma. of t he infant's testis: · ·An account of 15 oases, J . Bat h. & Bact. 80: 147-156, 1960.

9. Abell, z.r. R. and Holtz, F.: Testicular neoplasms · i n infants and children• · 1. Tumors of gern cell origin,. Cancer 16: 965-981, 1963··

10. Abell, M.R. and Holtz, Fo': Testicular neoplasms in adol escents, Cancer 17:881- 896, 196~.

n . Andere on, z.r.s·.: I•zyxop!.pil~ ependymomas presenting in the · so.ft tissue over the sacrococcygeal region, Cancer 19: 585- 590, 1966.

CASE 15 Wolman's Di sease .

1. Abramov, A., Schorr, S . and W.o.lman, 11; : Generalized xanthomatosi s with '-oal cified adrenals , A.Ir. A. Am. J . Dis. Child . 91: 282- 2861 1956.

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2. WolJlla.n, M. Sterk, v.v., Gatt , s. and Frenkel, M.: Primary familial xanthomatosis with involvament and calcification of the adrenals. Report of two more cases in siblings of a previously described infant, Pediatrics 28:742-757, 1961.

}. Crocker, A.c., Vawter, G.F., Neuhauser, E .B.D. and Rosowsey, A. 1 Wolman's disease: Tbreee new patients with a recently described lipidosis, Pediatrics 35:627-640, 1965.

1. Poore, T. N., Dock.erty, 1-f.B., Kennedy, :RL. J . and Walters·, w.: Abdominal neuroblastomas , Surg. Clin. N • .Ail!er. 1121-1141, August , 1951.

2. Horn, R. C. Jr., Koop, C.E. and Kiesewetter, W .B.: Neuroblastoma in childhood. Clinicopothologic study of forty-four cases, Lab. Invest. 5&106- 119, 1956.

} • Hanfi!Dan, 0 . F. and Girdna,y, B . R.I ·Tho roentgenographic findill8S associated 11i th neuroblastoma, J . Pediat. 51:621- 633, 1957.

4• Fox, F., Davidson, J . and Thomas, L.B.s Maturation of sym~thicoblastoma into ganglionelll'Oma. l!e'OO'>"t of :'. ~tients with 20 -

· and 46 - year survivals respeotive~ , Cllmcer 12:109- 116, 1959·

5· Gross, R.E. , :Earber, s . and Martin, L.W.: Neuroblastoma sym-I&theticum. A study and report of 217 cases, Pediatrics 23: 1179-1191, 1959 .

6. Voorhess , H. L. and Gardner, L. I .: Urinary excretion of nor-epinephrine, epinephrine and ;-methoxy-4-~o:x;yme.ndelic acid by children with neuroblastoma., J . Olin. Endoorinol. 21:321-335, 1961.

7• Dargeon, H.W.: Neuroblastoma, J . Pediat. 61:456- 471, 1962 .

a. Kontras , S.B. : Urinary excretion of 3Jilethooc;r- 4-eydro:x;yme.ndolic acid in children 1dth neuroblastoma , Cancer 15:979- 996, 1962.

9. Voorhees, M. L. and Gardner , L. I . : The valve of serial catechola-mine dete:xmina.tions in children with neuroblastoma. Report of a. case, Pediatrics 30:241-246 , 1962.

10. von Stu.dnitz, w., Kaser, H. and Sjoerdsma., A.: Spectrum of catecholamine biocbemi str,y in patient's with neuroblastoma, New Ellgl. J . t-Ied. 269-232- 235, 196;.

11. Koop, C.E. and Hernandez, J.R.: lieuroble.stoma: Experience with 100 cases in children, Surger,y 56:726-7};, 1964.

-10-

12. Goldman, R.L., Winter ling, .A. N. an

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12. :Burgert, E. o . Jr. and Glidewell, 0.1 Dactinomyoin in Wilms ' tumor, J.Al-!A 199:464-468, 1967.

CASE 18 Tubercu1osis.

CASE l9 Fogal nodular h.yperpJ.s.sia of' the liver.

1. Ka;yf s. and Talbert, P. O.: Adenoma of the liver., mixed type ~hamartoma). Report of two oases, Cancer, 3:307-3151 1950•

2. Christ opherson, ~I .H. and Co~lier , H. S.I ' Pri.mary benign liver-cell tumors in infancy and childhood, Cancer 6:853- 861, 1953•

; . Edmonson, H. A. : Differential diaenosis of tumors and t\li!IOrb -like lesions of the liver in infanc:{ and childhood, A. H. A. Am. J . Dis. Child. 91:168-186 , 1956.

4• Cleland, R. S .a Benign and malignant tumors of the liver, Pediat. Clin. N. Amer. 6:427-4471 1959.

CASE 20 Eosinollhillic m.nuloma. o£ bone.

1. Dargeon, Harold W. K.: Reticuloendotheliosea in Childhood. A Clinical Survey, Spring:f'ield, Illinois, Charles c. Thomas 1 Publisher, 1966, :PP• 127 •

2 . Enriquez, P. 1 Dahlin, D. c ., ~les1 A.Jl , l!.nd Hende;ruon1 E.D,; Histocytosis X: a clinical study, l'eyo Clin. P.roc. 42188-991 :1,967.

HOUSTON SOCIET;l OF CLINICAL PATHOLCXHSTS

196 7 SE!.f iNAR

PEDIATRICS, PATHOLOGICAL AND RADIOLOGICAL SEMINAR

April 22, 1967

9.:00 A.M. - 4:00 P.M.

a t

ST. LUKE'S EPISCOPAL HOSPITAL

Audit orium--Texas Chil dren's Hospital

Texas Medical Center

Houston, Texas

Supported by

ST. LUKE'S EPISCOPAL HOSPITAL and the AHERICAN CANCER SOCIETY

Conducted by

J.4MES B. AREY, M.D. Professor of Pathology

Temple University School of Medi cine Philadelphia, Pennsylvania

JOHN A. JORKPATRICK, JR., M.D. Prof essor of Radi ology

Temple University School of Medicine Philadelphia , Pennsylvania

DIAGNOSES

Please mail. your diagnoses in ad;vance· or the meeting in order to allow ample time· for tabulations . Signat ure is not required. Registration f or the meeting is not required. The meeting will start promptly at 9:00 A.M.

l.

2.

3·

4.

5·

6.

7·

8.

9·

10.

u .

12.

13.

14.

16.

17.

i8.

19·

20.

Please mail t o: William T. Hill, M.D. St . Lu.lre • s Episcopal Hospital Texas Medical Center Houston, Texss 77025

--~ ...

HOUSTON SOCIETY OF CLINICAL PATHOLOGISTS - 1967 Seminar

CASE 1. 4f year old W/M

Contributed by Dr. Harvey S. Rosenberg, Texas Children' s Hospital, Houston, Texas.

Chief Complaint: Repeated bouts of pneumonia for three and one-half years . At 3 years, hospitalized because of upper respiratory infection .

Physical Examination: Pale and undernourished with purpuric lesions over both lower extremities . Hemoglobin 4. 8 gms. which rose to 7·5 gms. after transfusion . Clotting and bone marrow studies were normal. Jr-Rsys: Pneumoni t i s of the right upper lobe.

Subsequently, there was persistence of x-ray changes in the right upper lobe. Hemoglobin again dropped to 4.8 gms. After a total of eight blood transfusions over several months, he became jaundiced. Both the liver and spleen were enlarged. Serum bilirubin was 11 mg. and the thymol turbidity was 16 units . A reticulocyte count was 8~.

Six weeks later, the Jaundice cleared. Purpura and thrombocyto-penia occurred.

At 4 years, a Coomb's test was positive. This had been performed before and had always been negative. A reticulocyte count at this time was 30~. The platelet count was 18,000. Because of the persistent right upper lobe pneumonia, he was taken to surgery for removal of the upper and middle lobes of t he right l ung.

DIAGNOSIS: _______________________ _

C~; __________________________________________ ___

CASE 2

HOUS'ION SOCIETY OF CLINICAL PATHOLOGISTS - 1967 Seminar

CASE 2. 10 veek old W /~1

Chief Complai nt:

Contributed by Harvey S. Rosenberg, M.D. , Texas Children's Hospital, Houston, Texas

Cyanosis, hyperpnea and coughing.

In the hospi tal, the i nfant continued to cough considerable amounts of vhite mucus. Chest x-ray: Marked haziness 1n both l ung fields vith the heart borders partially obscured. E.C.G.: Right ventricular hypertrophy . Ear oximetry: A saturation of 7131> 'With room air and 99i vhen breathing oxygen. Cardiac catheterization : No heart anomaly but the pulmonary artery pressure was increased. Skin tests f or tuberculosis and 1\mgi vere negative. A lung biopsy was performed three weeks after the infant was admitted t o the hospital . Following this, steroid therapy was started with good response initially, but his breathing difficulties resumed 'Within a veek. Steroids 'Were given over the next five 'Weeks, but he lost veight and developed diarrhea. Terminally, the diarrhea became grossly bloody and his hemoglobin dropped precipitously. The breathing difficulty persisted until the time of his death at four months of age.

DIAGNOSIS:_--'------------------------

COMMENT:

CASE 3


CASE 3· 5 year old W/M Admitted 5/17/57 Expired 5/22./57

Contri but ed by Dr. James B. Arey, St. Christoper 's Hospital f or Children, Plliladephia, Pennsylvania

Diagnosis of leukemia i n 1956. He responded well to methotrexate and 6 mercaptopurine. In April 1957, he developed measles . The rash disappear ed after four days. He remained intermittently febrile and a cough persisted.

X-ray on 5/10/57: Diffuse and patchy infiltrate6 distributed throughout both lungs, with peribronchial inflammatory change.

Physi cal Examination: Temp. 104. Reap. 24. A cachectic, chronically i l l pati ent . The skin was dry and there were petechiae in both inguinal regions . ¥..oist rales over both lungs. The tip of the spleen 1.5 em. below the left costal margin and the liver 1.5 em. below the r~ght costal margi n.

Hgb. 12 .1 gms.%, WBC 5, 300 with normal differentia.! and platelets . Bone marrow aspirate: Normal. He received penicillin and tetracycline but his temperature continued to be elevated 102 to 104. Paroxysmal cough and r espiratory distress progressed . On 5/21/57 subcutaneous emphysema of the neck was apparent and rapi dl y spread t o face, chest, and abdominal wall.

D~NOSIS: ____ ~-----------------------------------------

co~~:--------------------·--------------------------

~.--· / - 2. 2. - os

HOUSTON SOCIETY OF CLINICAL PATHOLOG!BrS - 1967 Seminar

CASE 4. 2 year old C/F Admitted 1/ 22/65

Contri buted by Dr. James B. Arey, St . Christopher's Hospital for Children, Philadelphia, Pennsylvania

Chief Complaint : Fever and cough which occasionally led t o vomiting - f our days duration.

Past History: Full term. Normal gestation, labor and delivery. Birth weight: 4:ibs . and 4 ozs . Always small f or age, and a poor eater.

Physical Examination: Temp. 101. P. 32. Reap. 6o. A thin, undernourished felliBle. Height was 33i inches, in the lOth percentile for her age. Weight - 17i lbs . , considerably below the third percentile. Slight flari ng and ret raction were noted with respirations . Slight hyperresonance over the left hemithorax. Where breath sounds were decreased, scattered crackling rales were audible in the left hemithorax posteriorly near the midline. The remainder of the examination was normal.

Hgb. 9·7 gms ., WBC ll,200, 54 neutrophiles and 46 lymphocytes. The ur:inalyses, blood culture, nasopharyngeal culture and sweat chlorides were within normal limits. BronchOI!COpy revealed narrowing of the left main bronchi al orifice as if by extrinsi c pressur e; coli form and aerobacter organisms were grown from the bronchial secretions.

Previous x-ray: Six months before admiss i on, cysts were present i n the left l ung.

After oral penicillin and sulfadiazine, she became afebrile. On 2/23/65, the left lower lobe of the lung was removed.

DDWN~IS: ·~------------------------------------------

CO~T=----------------------------------------------

,3 - s~


~· W7F1ilfant Born: 3/ll/59 Admitted: 3/13/59 Died: 3/15/59

Contributed by Dr. James B •. Arey, St . Christopher's Hospital for Children, Philadelphia, Pennsylvania

A normal gestation and delivery. Cyanosis at the time of birth. Respiratory distress became progressively more severe and hospitalized at the age of two days.

Physical Examination: Weight: 7 lbs. 2 ozs. Resp. 56. P. 124. A deeply cyanotic, weak, hypotonic infant. The lungs clear to auscultation. Grade 2 to 3 harsh systolic murmur heard best along the left lower sternal margin. Femoral pulses were weak. The liver was palpable 4.0 em. below the right costal margin. The spleen 1.0 - 2.0 em. below the lett costal margin. No peripheral edema.

Hgb. 19.4 gma., WBC 14,ooo, 72 neutrophile& and 28 lymphocytes; one normoblast per 100 white blood cells.

In spite of digitalis, oxygen therapy and gavage feedings, the infant continued to have severe dyspnea, cyanosis, tachypnea and tachy-cardia. Death occurred 48 hours after admission.

DIAGNOOIS: ______________________ _

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CASE 6

I


CASE 6. WfM Infant Born 9/4/57

Contributed by Dr. James B. Arey 1 St. Christopher's Hospital for Children, Philadelphia, Pennsylvani a

Born after 45 weeks gestation. Weight: 6 lbs . and 15 ozs. Birth appearance of a "little ol.d man" with mild desquamat ion of the skin. The only complication of pregnancy vas Herpetic vulvitis at eight and one-half months.

On the third day of life he became febrile and developed severe respiratory difficul.ty. A catheterized specimen of urine revealed pus cells with coliform staphylococci. He responded t o antibiotics. Discharged f rom the hospital at the age of four and oneftbalf weeks . (Weight: 7 lbs. 6 ozs.)

Readmitted three days later with cough, coryza, and fever of 104°. No response to Rx. Transferred to St. Chr istopher' s.

Physical Examination: Temp. l.o4. P. 210. Resp. 80. A critically ill infant. Skin pale and thin. Almost no subcutaneous tissue, but good turgor. The hair on the scalp vas sparse, no eyebrows, very few eyelashes . Breath sounds were decreased and there wer e few rales over t he right upper lobe.

Hgb. 8. 2 gms. , WBC 211000, 54 lymphocytes, 19 neutrophile a, 2 bands 1 8 metamyelocytes, 2 myelocytes , 2 eos inophiles, 6 "blast" forms and 3 plasma cells. Subsequently, the WBC returned t o normal. Urinalyses were normal and blood cultures sterile . Duodenal drainage bad 100~ trypsin activi ty. In an attempt to deter mine the chlori~e l evel of sweat, the temperature rose from 99.2 to 102.4 in 45 minutes but no sveat appeared. Nasopharyn-geal and bronchial secretions yielded staphylococcus aureus, coagul.ase positive . T.B. and Risto. skin t ests - negative.

Rx consisted of oxygen, humidity, antibiotics, intravenous fluids, blood transfusions and vas feeding by nasogastric tube. No weight gain occurred. Terminally, there was severe respiratory distress and temperature of 102.

DllGNOSm: __________________________________________ ___

CO~:----------------------------------------------

HOUS'ION SOCIETY OF CLniTCAL PA'l'IIOLOOISTS • 1967 Seminar

CASE 7· 9 year old C/M Admitted 3/ 23/ 66

Contributed by Dr. James B. Arey, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania

Chief Complaint: SWelling in the neck, first noted the preceding day by his school teacher. The child was e.ntirely asymptomatic.

Physical examination reveal ed four small freely moveable lymph nodes in the left posterior cerVical chain. The trachea was slightly deviated to the rigllt. A painless, hard, l obular, moveable mass 3.0 x 5. 0 em. extended from the left supraclavicular area across the midline. There was no limitation of movement of the neck.

Hgb. 13. 3, WBC 4, 750, 6o neutrophiles, 39 lymphocytes and l eosinophile. The red blood cells: slightly hypochromasia, anisocytosis, occasional target cells, and stippled cells. Urinalysis was normal.

A biopsy of the cervical mass was performed. Subsequently, the cervical as well as the mediastinal mass was removed.

D~QNOOIS: __________________________________________________________________________________ ___

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HOUSTON SOCIETY OF CLINICAL PATHOLOGISTS • 1967 Seminar

CASE 8. 4 month old W/M Admitted 1/27/66

Contributed by Dr. James B. Arey, St. Christo~her's Hospital for Children, Philadelphia, Pennsylvania

Referred because of roentgenographic evidence of a large mass in the right hemithorax.

Delivery weight: 8 lbs . 12 ozs. "Noisy" respirations were noted a few days after delivery and had persisted. He ate well and gained to a weight of 10 lbs . (6 weeks). Two months of age he developed a cold associated with cough and fever; these promptly disappeared wit h treat-ment. The "noisy" respirations continued . A physician noted dullness over the right side of the chest and x-ray revealed a lsrge mass in the right hemithorax.

Fnysical Examination: Temp. 101.2. P. 18o. Resp. 72. B.P. 102/58. A chronica.lly ill male infant with slight suprasternal and infracostal retract ions with respiration . The right hemithorax was dull to percussion and breath sounds were decreased. Wheezes and scattered rhonchi were audible in both lung f ields . The liver extended 5.0 em. below the right costal margin in the midclavicular line. The remainder of the examination vas non-contributory.

Hgb. 12 .4 gms. , WBC 19,000, 62 neutrophiles. Urinalysis was normal .

The mass was exci sed with the attached seventh and eigth ribs .

DIAGNOSIS: _______________ ________ _

OOM~=-------------------------------------------

HOUSTON SOC IETY OF CLINICAL PATHOLOGISTS - 1967 Seminar

CASE 9· 3 month old W/F Admitted ll/9/65

Contributed by Dr. James :B. Arey, St. Christopher's Hospital f or Childr en, Philadelphia, Pennsylvania

Chief Co!liPlaint : "A lump on the t op of her head." Thi s was first noticed by the father one month ago. There vas no history of trauma or associated SYDIPtoms.

Physical examination was negative except for the presence of a firm, r ai sed bony l esi on 1 . 5 em. in diameter, just anterior to t he patent anterior fontanelle .

Hgb. 10. 8 gms. , WBC l0,4oo, 77 lY!liPhocytes, 20 neutrophiles, 2 monocytes and l eosinophile .

The tumor , vhich involved the dura, vas excised on 11/ll / 65. Some sections are from a similar tumor in the mediastinum of a seven month old infant. This material was kindly submitted by Dr. Kazuald. Misugi, The Children's Hospital, ColUJilbus, Ohio.

DIAGNOSIS: ______ ____ ________ _____ _

ro~: _______ ___________________________ __


CASE 10. 9 year old W/M

Contributed by Dr. James B. Arey, St . Christopher's Hospital for Children, Philadelphia, Pennsylvania

Chief Complaint: Recurrent epistaxis for the past four months; the bleeding usually occurred predominantly from the left nostril. On the night prior to admission four episodes of epistaxis occ.urred. T&A two years before admission, at which time a right myr ingotomy had also been performed. There vas a history of recurrent sore throats, of several bouts of pneumonia and of a chronic postnasal discharge. He vas said to be allergic to ragweed.

Physical. Examination: Temp. 96. P. &:>. Reap. 20. B.P. 120/70. An alert boy in no acute distress. The right ear drum appeared somewhat scarred. The mucosa of the right nostril vas boggy and somevbat blue. The left nostril was filled with mucus and blood. No polyp could be seen. The remainder of the examination was normal.

Hgb. 12.4 gms., WBC 6,000, 71 neutrophiles, 28 lymphocytes, and 1 eosinophile. The platelet count and urinalysis were normal.

The mass exci sed.

DI.~GtiOSIS: ____ __________________ _ _

00~:---~~-------------------------------------

CASE 11

- , ~- - (!o ft


CASE ll. 10 year old M Admitted 11/3/66

Contributed by Dr. James B. Arey, st. Christropher's Hospital for Children, Philadelphia, Pennsylvania

Chief Complaint: Persistent eneuresis over the past few years. In addition, frequency and occasional bouts of dysuria had been noted during the past few months. In the past six months he had a brief hospitalization. Elevated blood pressure and possible agenesis of one kidney recorded.

Physical Examination: Temp. 99 . P . 8o. Resp. 20. B.P. 150/120. An alert, well-developed child. The femoral pulses. were palpable and strong bilaterally. There were no cardiac murmurs or enlargement . A r ight inguinal hernia.

Hgb. 14.6 gms. WBC 9,100, 44 neutrophUes, 47 lymphocytes and 9 eosinophiles. Repeated urinalyses were normal. BUN was 12. 5 mgs.i. Calcium 10, phosphorus 4 . 4 and alkaline phosphatase 6.9. The VMA level was normal. An I . v. nephrogram revealed a normal right kidney. Let't kidney: Small amount of contrast medium in t wo very small calyces at the upper half. A voiding cystourethrogram was normal. Spina bifida occults at L5. Renal arteriogram: Right kidney normal. The left kidney was very small, located higher than is usual, with no well defined arterial supply. Over a period of time a let't "nephrogram" appeared.

On 11/23/66 the left kidney was excised. By 11/29/66 the blood pressure was normal.

DIAGNOSIS: ______________________________________________ _

co~~:--~---------------------------------------------

II • • 'f • 6.;


CASE 12. 5 year old W /F Admitted 10/20/65

Contributed by Dr. J ames B • . Arey, St. Christopher 's Hospi t al for Children, Philadelphia, Pennsylvania

Chief Comwlaint: Intermittent svelling of the ankles which subsided spontaneously - duration 3 months. Two weeks prior to admission she developed a cough and became short of breath . Paroxysmal nocturnal dyspnea and generalized edema had been present for one week.

Physical Examination: Temp. 100 . P. 150. Reap. 80. B.P. l 6o/l l0. An edematous, well-developed child with severe respiratory distress . The above blood pressure vas taken in the right arm but vas unobtainable in the l eg. The radial pulses were weak. The femoral pulses were not palpable. Rales and wheezes were audible throughout both lungs . A questionable FMI in the left anterior axillary line. A grade 3 systolic murmur at the apex . The liver vas 4.0 - 5.0 em. below the right costal margin. There was 4+ edema of the lower extremities.

Hgb. 12.5 gms . , WBC 27,300, 79 polys, 1 bands, 14 lymphocytes. The WBC count averaged about 15,000 with 70-95'1> polys. Urinalysis: 2-3+ albumin. 25··30 RBC's per H.P. J at times only rare RBC and 40-50 WBC's. ASO Titer 125 Todd Units, C-reactive protein 2+. BUN varied from 9 ·4 to 31.5 mgms .

Cardiac Catheterization: Catheter inserted into the right femoral artery could not be advanced beyond the level of Le· Accomplished via the right brachial artery. Multiple areas of narrowing of the aorta, filling defect in the abdominal aorta. Several mitral stenosis. Inadequate r enal arterial supply, more marked on the r ight aide. Bl ood pressure in the upper extremities was consistentl y elevated and normal in the l ower extremities. A r i ght nephrectomy was performed. Pied the second postoperative day . Sect ions from the l ef t common carotid artery.

DIAGNOSIS: __________________________________________________ __

COMMENT:_---:------ -------- -------

HOUSTON SOCIEI'Y OF CLINICAL PATHOLOGISTS - 1967 Seminar

CASE 13. 10 month old W/F

Contributed by Dr. Harvey s . Rosenberg, Texas Children's Hospital, Houston, Texas

At the age of nine months , this white female in:t"ant had clitoral hypertrophy. She was seen by a gynecologist who interpreted the findings as phimosis, enlargement of the right labium majorum, and minimal clitoral enlargement . She was also seen by an endocrinologist who agreed that there was no growth or endocrinologic problem. The in:t"ant was circumcised, a part of the enlarged labium was removed and an i mper:rorate hymen was incised.

One month later fresh blood was noted in her vagina, followed shortly by acute urinary retention. An IVP and Cystogram revealed no renal abnormality. A large soft tissue pelvic mass was ide.ntified. By Cystogram, the bladder was elevated and displaced superiorly and anteriorly by the soft tissue mass. The mass was pushing down against the rectum.

A supr apubic exploration of the pelvis was performed followed by biopsy and subsequently pelvic exenteration. At the time of surgery no t umor was identified beyond t he vagina. Lymph nodes were negative for metast asis .

Five months later there was recurrence of a non-tender mass at the lower end of the abdominal incision.

DIAGNOSIS : _ ______________________ _

COMMENT: _______________________ ___

CASE 14


CASE 14. 2 month old W/F Admitted 7/14/66

Contributed by Dr. James B. Arey, St . Christopher's Hospital f or Children, Philadelphia, Pennsylvania

The infant began to fret and cry whenever she was placed i n a s itting position; at that time only slight puffiness could be noted about the base of the spine. One month later, a mass "about t he size of a walnut" was palpable over the coccygeal region and a distinct dimple was present in this region.

Physi cal examination negative except for sacral mass .

Hgb. 10.2 gms ., WBC 9,900, 67 lymphocytes , 30 neutrophil es and 3 eosinophiles . Urinalysis: Trace to l+ albumin, 12-20 WBC per H. P. Intravenous urogram, roent gen f ilms of the chest and a skeletal survey r evealed no abnormalit ies . A soft t issue mass in relation t o the distal end of the sacrum. Areas of calcification were present within the mass , which on barium study of the colon was seen t o displace t he rectum anteri orly and t o the left; the mass appeared to extend to about the level of S2.

The mass was excised on 7/18/66.

DIAGNOSIS: _ _ _ _ _ _ _________________ _

CO~:--~-----------------------

CASE 15

HOUSTON SCX:IETY OF CLINICAL PATHOLOGISTS - 1967 Seminar

CASE 1 • W M Infant Admitted at 2 days old Died at 2t months


Birth weight: 7 lbs. and 3 ozs. He was admitted to the Hospital because two female siblings had died in the first several months after birth (hepatomegaly, splenomegaly, adrenal calcification and failure to thrive). The first born sibling, a male, was living and well. Roentgen examination of the abdomen of the present infant on the first day of life had demonstrated bilateral adrenal calcifications.

Physical Examination: P. 16o. Resp. 48. A veil-developed and vall-nourished infant . The pulse rate was 16o per minute and the respiratory rate 48 per minute. The liver was 2 em. below the costal margin and the spleen was not palpable. The Moro reflex was active.

The initial hemogram, urinalysis, electrolytes, serum protein, A/G ratio, serum immunophoretic pattern, and urinary amino acid were normal. EEG at one month of age, was normal.

X-Rays: A normal skull, long bones, heart and lungs.

Initially, fed well and gained weight steadily . The fourth day: The spleen was palpable 3 em. below the left costal margin. Bone MarrO'>~: Vacuolated h1st1ocytes. The tenth day: Stools were intermittently large and loose. Severe edema vhich persisted until death. The third week: Pale, vaxy, pallid skin. Splenomegaly and hepatomegaly were progressive. Terminally 1 the spleen extended into the pel vis . Serum cholesterol abnormally high. The eighth veek: Spiking fever, tachypnea, and some respiratory retractions. Intake decreased below caloric requirements, bleeding occurred from needle puncture. Platelet count 50,000. Lympho-cytes vacuolated . The tenth week: Losing weight, melena and severe respiratory distress . Xylose and Vitamin A absorption test - abnormal. Myelocytee and metamyelocytes in peripheral smear. Icterus, bradycardia and hypothermia developed terminally.

DIAGNOSIS: ______________________________________________ _

CO~=----------------------------------------------

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CASE 16 . 17 day old W/F Admitted 4/3/57

Contributed by Dr. James B. Arey, St. Christopher's Hospital for Children , Philadelphi~ , Pennsylvania

Normal gestation , labor and del ivery. Birth weight: 8 lbs. 3 ozs .

Chief Complaint: A large mass was noted in the lateral aspect of the r ight side of the neck at t he time of deli very.

Physical Examination: Weig)lt: ·8 lbs. 10 ozs. Well devel oped, well nourished infant in no distress. A hard, nodular submandibular mass 6 x 4 em. , not adherent to the overlying ski n, was present in the lateral aspect of the r ight &ide of the neck; t his pushed up the inferior aspect of the right ear. The trachea appeared to be in t he midline. The remainder of the examination was normal.

Hemoglobin, white blood cell count and different ial, as well as bone mar row, were normal. The urine contained a trace of albumin but was otherwise normal.

X-Ray: Chest and skeleton were normal. No deviation of the trache.a or of the barium- filled esophagus.

The mass was removed on 4/5/57·

DIAGNOSIS : _____________________ _ _ _

co~~=---------------------------------------------

CASE 17


CASE 17. l year old W/M Admitted 4/21/65

Contributed by Dr. James B. Arey, St . Christopher' s Hospital for Children, Philadelphia, Pennsylvania

A normal i nfant on routine physical l2/l5/q4. A routine examination on 4/20/65, a large mass was found in the right upper quadrant and the right flank.

Physical examination revealed a healthy male infant with no abnormalities except for a firm, somewhat nodular mass in the abdomen extending into the right flank . Both testes were descended.

Urinalysis : Sp. gr. 1 .010, l+ albumin and 3+ acetone, sediment normal. The hgb. ll gms., WBC 8,900, 48 neutrophiles and 52 l ympho-cytes. Bone marrow aspirate normal . X-Rays: Chest and skeleton were normal except f or mild osteoporosis involving the proximal end of each humerus. An inferior vena cavagram revealed filling of Batson's plexus with no visualization of the inferior vena cava. Both renal collecting systems were Visualized and there was some distorti on of that on the right.

On 4/23/65 , a right nephrectomy.

DIAGNOSIS: ______________________________________________ _

CO~:------~--------------------------------------

HOUSTON SOCIETY OF CLINICAL PATHOI.OOISTS - 1967 Seminar

CASE 18. 4 year old C/M Admitted 6/10/66


The mother bad noted a "large stomach" since be bad been tvo yea.rs of age, but a bulge 1n the left side vbUe walking vas first noticed two weeks before admission. There was no history of abdominal pain but be often held his abdomen while walking and often had to rest.

Physical Examination: Temp. 96.4. p, 8o . Reap. 20. B.P. 105/70. A well nourished infant in no apparent distress. An obvious mass was present in the left side of the abdomen. The mass was firm, non-tender, filling almost the entire left side of the abdomen. The remainder of the examination was essenti ally negative.

Hgb. 10.0 gms . , WBC 6,650, 54 lymphocytes , 39 neutrophiles, 6 eosinophiles and 1 monocyte. Urinalysis was normal.

At operation (6/11/66) a large mass which extended from the rib cage to the pelvis displaced the left half of the colon medially. The left kidney was at the superior pole of the mass . The mass was cystic and whitish fluid exuded. The cyst collapsed. The sections are from the wall.

DIAGNOSIS: ____________________________________________ ___

CO~:----~~--------------------------------------

HOUSTON SOCIETY OF CLINICAL PATHOLOOISTS - 1967 Seminar

CASE 19. 6 year old lo',de Admitted l/12/65


Chief Complaint: A mass in the abdomen. Thi s had "been first noted eight days earlier when he had been seen as an outpatient because of mumps .

The patient had been l argely asymptomatic and had developed normally. Occasional pains in t he right quadrant apparently brought on by rapid changes of position . Occasional vomiting during past week. His appetite was good . llo icterus. No change in the st ools .

Physical Examination : Temp. 98.2 . p, 84. Resp. 24. A small child in no apparent distress. A firm, nodular, non- tender mass which moved with respirations extended 8.0 em. "below the costal mar gin in the right midclavicular line thought to be liver. The spleen non-palpable.

Hgo. 11.6 gms . WBC 9 , 300, 64 neutr ophiles, 27 lymphocytes, 8 eosinophiles and 1 monocyte . Urinalysis: l+ albumi nuria. Bil i rubin was 1.1 mgs.~ with 0.2 direct. Total protein 6.7 gms . A/G 3·9/2.8 . Prothrombin time 100~, cephalin flocc. 3+, thymol flocc. 1+, and thymol t urbidity 2.5 unite. Alkaline phosphatase was 5.4 units. Intravenous pyelogram: No distortion nor displacement. Oral chole-cystogram: A gallbladder of normal size and shape, a suggestion of a soft tissue mess in i ts vicinity; on the lateral projection, the gallbladder appeared to be indented anteriorly.

Operated on l / 18/65 .

DIAGNOSIS : ____________________________________________ ___

CO~'----------------------------------------------


CASE 20. 12 year old C/F Admitted ll/18/66


Chief Complaint: Gradual, relatively painles s, swelling over the right side of the mandible of about one month's duration. Denies trauma.

Past History: T&A June •66. A systolic murmur at the apex bad been recorded. Oral prophylactic penicillin since June.

Physical Examination: Temp. 98.4. P. 92. Reap. 20. B.P. l20/6o. The teeth in good repair. A non-tender swelling was present over the medial portion of the right side of the mandible. A grade 4, harsh systolic murmur was audible in the 5th interspace in the lett midclavicular line. No other abnormalities were noted.

Hgb. 12 .5 gms. WBC 8,8oo, 50 neutrophiles, 46 lymphocytes, 3 eosinophiles and 1 monocyte. Sedimentation rate 35 mm. per hour. Serum calcium 10. 3, phosphorus 4.4, and alkaline phosphatase 9.8. Urinalysis was normal.

X-Rays : Skeletal survey revealed no other abnormalities.

A biopsy of the mandibular lesion on 11/19/66.

DIAGNOSIS: ____________________________________________ ___

co~~: ______________________________________________ ___

1967 SEMINAR==~~ · 2015. 10. 3. · ~===1967 seminar==~~ houston society of cunigal pathologists . pediatrics, pathological and radiological seminar james b. arey, m.d. professor

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