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病理診断生涯教育プログラム Performance Improvement Program in Surgical Pathology JPIP ◆ JPIP1
Case # 2019-01 症例 # 2019-01 Site: Left kidney Clinical Summary An otherwise healthy 2-year-old girl is taken to the pediatrician after her mother notes a relatively sudden increase in abdominal girth. Upon physical examination, the pediatrician palpates a large abdominal mass and orders a series of tests, including a whole abdomen computed tomography (CT) scan. The CT scan confirms the presence of a heterogeneous cystic left renal mass with enhancing soft tissue and adipose tissue components, and excludes evidence of lymphadenopathy and/or metastatic deposits. The patient undergoes surgery. The well-circumscribed intrarenal mass involves the upper pole of the left kidney, measures 9.0 x 7.0 x 6.0 cm, and exhibits a variegated pink-tan, soft, and friable cut surface; there are areas of hemorrhage involving 20% of the cut surface, as well as patchy yellow-tan and soft areas. Master List of Diagnoses (2388) Cellular mesoblastic nephroma (1020) Cystic partially-differentiated nephroblastoma (5596) Intrarenal teratoma (1666) Metanephric adenoma (5597) Nephroblastoma with heterologous elements (teratoid
Wilms’ tumor) (1012) Neuroblastoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following is true regarding the term
nephroblastoma with heterologous elements (teratoid Wilms’ tumor)? (6016) It can only be used to designate extrarenal tumors
with mature heterologous epithelial and stromal differentiation.
(6017) It identifies a subtype of Wilms tumor that is immunonegative for WT1.
(6018) It is synonymous with fetal rhabdomyomatous nephroblastoma.
(6019) It is used to designate blastema predominant Wilms tumors.
(6020) It is used to designate Wilms tumors when the heterologous epithelial and stromal differentiation comprise >50% of the tumor volume.
2. In cases of Wilms’ tumor, which of the following findings
has been shown to be associated with more clinically aggressive disease? (2912) MYCN amplification (6021) Diffuse anaplasia (6022) t(12;15)p13;q25 chromosomal translocation (2028) Homer Wright rosettes (5017) BRAF V600E mutation
3. Which of the following genetic alterations can be seen in
association with Wilms’ tumor? (6023) Loss of imprinting on 11p15 (3613) N-myc amplification (6024) Parental isodisomy of chromosome 11p15.5 (6025) t(8;14) (6026) t(12;15)(p13;q25)
Case # 2019-02 症例 # 2019-02 Site: Ovary Clinical Summary A 41-year-old woman presents with abdominal distention, abdominal pain, and increased urinary frequency. A computed tomography scan shows a 12.0 cm diffusely hypoechoic right pelvic/ovarian mass. The patient undergoes right salpingo-oophorectomy. Grossly, the tumor is well-circumscribed and firm with lobulated, grey-white, glistening surface. The cut surface of the tumor is white with some areas of hemorrhage. Master List of Diagnoses (2081) Adult granulosa cell tumor (1711) Brenner tumor (1957) Endometrial stromal sarcoma (2540) Fibroma (1100) Fibrothecoma (1646) Leiomyoma (1697) Massive ovarian edema (2548) Thecoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. To which of the following ovarian tumor categories does
Clinical Summary A 6-month-old baby boy presents to an outside hospital with cardiomyopathy of uncertain etiology. The baby is transferred to a tertiary care center where cardiac transplantation is performed. Representative sections of the explanted heart are submitted. PAS stains with and without diastase show no increase in intracytoplasmic glycogen or increase of PAS-positive diastase resistant material. A modified Gomori trichrome stain shows granular staining of the cytoplasm. Diagnostic electron microscopy reveals increased numbers of an abnormal organelle. Master List of Diagnoses (5598) Arrhythmogenic cardiomyopathy (5599) Glycogen storage disorder (5600) Mitochondrial cardiomyopathy (5601) Mucopolysaccharidoses (1122) Myocarditis Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. A mother brings her child to the pediatrician stating that the
child is easily tired. The child exhibits developmental delay. Cardiac examination reveals a hypertrophic cardiomyopathy. A cardiac biopsy shows vacuolated myocytes that are PAS positive. Acid maltase activity levels were normal. Which of the following is the best diagnosis? (6034) A mitochondrial disorder (6035) Arrhythmogenic cardiomyopathy (6036) Type IIa glycogen storage diseases (Pompe
Site: Kidney Clinical Summary A 50-year-old man presents with longstanding hematuria and flank pain, and a 9.5 x 8.5 x 6.8 cm right renal mass with retroperitoneal lymphadenopathy is identified on computed tomography scan. The patient undergoes radical nephrectomy, where a similarly-sized white-tan firm renal mass is found invading the pelvicalyceal system, renal sinus, and perinephric tissue, with a renal vein thrombosis. A total of 34 of 45 regional lymph nodes are involved by metastatic tumor. The adrenal gland is present and uninvolved. On immunohistochemistry, the tumor cells are negative for multiple keratins and melanocytic markers. Tumor cells mark with CD10, P504S, TFE3, and CA-IX Master List of Diagnoses (2632) Clear cell papillary renal cell carcinoma (1665) Clear cell renal cell carcinoma (5602) Multilocular cystic renal cell neoplasm of low malignant
potential (1797) Papillary renal cell carcinoma (5603) TFE3-associated renal cell carcinoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following statements is true regarding TFE3-
associated renal cell carcinomas (RCCs)? (6049) Generally speaking, TFE3 RCCs do not have
necrosis or other high-grade features. (6050) TFE3 RCCs are less aggressive than TFEB RCCs. (6051) TFE3 RCCs may mimic clear cell RCCs. (6052) TFE3 RCCs show morphologic, immunophenotypic,
and behavioral uniformity. (6053) TFE3 RCC spread is primarily lymphatic.
2. Which of the following statements is true regarding TFE3
fusion tumors? (6054) ASPL fusion tumors are part of the melanotic
metastases than PRCC fusion tumors. (6056) PRCC fusion tumors show more psammoma bodies
than ASPL fusion tumors. (6057) PRCC fusion tumors show higher rates of cathepsin
K expression than ASPL fusion tumors. (6058) TFE3 fusion partners include PRCC, ASPL, SFPQ,
and MALAT1. 3. Which of the following statements is true regarding ancillary
testing for TFE3-associated tumors (immunohistochemistry, polymerase chain reaction [PCR], and fluorescence in situ hybridization [FISH])? (6059) FISH using a TFE3 fusion probe is the preferred test
due to lack of issues with fixation and/or incubation times.
(6060) Reverse transcriptase PCR is an unreliable method of diagnosing TFE3 RCCs.
(6061) TFE3 immunophenotype is variable and depends on the fusion partner.
(6062) TFE3 immunostain consistently detects expression in TFE3 tumors and is the test of choice for diagnosis.
(6063) TFE3 RCCs consistently express Melan A, HMB45, and cathepsin K.
3. TFE3関連腫瘍に対する補助的検査に関して正しい記載は、次のうちどれか(免疫組織化学、PCR、および FISH 法) (6059) TFE3融合プローブを使用する FISH は、固定および/
またはインキュベーション時間に関する問題がないために好ましい検査である
(6060) RT-PCRは TFE3 RCCを診断する信頼性の低い方法である
(6061) TFE3免疫表現型は様々であり、融合パートナーに依存する
(6062) TFE3免疫染色は TFE3腫瘍で一貫して発現しており、診断に最適な検査である
(6063) TFE3 RCC は一貫して Melan A、HMB45、およびカテプシン K を発現する
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Case # 2019-05 症例 # 2019-05 Site: Liver Clinical Summary A 70-year-old man presents with vague abdominal pain and increasing jaundice. His past medical history is notable for chronic hepatitis C. A computed tomography scan reveals a left lobe mass in the liver. Gross examination of the hepatic lobectomy specimen reveals a cirrhotic liver with a tan soft 9.0 cm mass. Of note, the gallbladder and extrahepatic bile ducts are unremarkable on gross examination and histologically. Master List of Diagnoses (5604) Dysplastic nodule (2484) Focal nodular hyperplasia (2485) Hepatocellular carcinoma (2277) Intrahepatic cholangiocarcinoma (1518) Macroregenerative cirrhotic nodule Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following is correct regarding hepatocellular
carcinoma (HCC)? (6064) Clonorchis sinensis and Opisthorchis viverrini
infection are important risk factors. (6065) HCCs are more frequently encountered in males and
in African Americans. (6066) Portal tracts are readily identified within the tumor. (6067) The lesion develops as result of shunting of arterial
blood flow, which leads to the production of fibrous bands around nodules of hepatocytes.
(6068) Tumor grade is a strong prognostic factor. 2. Which of the following is correct of the fibrolamellar variant
of hepatocellular carcinoma? (6069) Neoplastic cells with eosinophilic cytoplasm with
frequent pale inclusions and cytoplasmic globules grow between dense fibrous septae.
(6070) Small cell change is a prominent feature. (6071) The tumor is distinguished by CK7- and CK19-
positive glands of cuboidal cells embedded in fibrotic stroma.
(6072) The tumor is comprised of regenerative hepatocytes arising in a background of cirrhosis with no expansion of the hepatic plates.
(6073) The tumor is the result of shunting of arterial blood flow, which leads to the production of fibrous bands.
3. Which of the following are histologic features of a dysplastic
hepatic nodule? (6074) Collections of hepatocytes with a decreased cell
volume, minimal nuclear pleomorphism, an increased nucleus to cytoplasm ratio, and increased nuclear density
(6075) Entrapped portal tracts with hepatocytes sharing the same histologic features as those in adjacent parenchyma
(6076) Expanded and disrupted hepatic plate architecture with neoplastic cells growing in solid, trabecular, and pseudoglandular patterns
(6077) Marked nuclear pleomorphism, marked hyperchromasia, and anaplastic giant cells
(6078) Nodular expansion of hepatocytes with intervening thin fibrous septae
Case # 2019-06 症例 # 2019-06 Site: Lung Clinical Summary A 70-year-old woman with a long history of smoking presents with weight loss, cough, and shortness of breath of several months’ duration. A chest radiograph shows a large dense opacity in the right upper lobe with traction of the minor fissure and hilar adenopathy. Computerized tomography (CT) scan reveals a 12.0 cm mass with enlarged hilar lymph nodes. Mediastinoscopy is negative. Right upper lobectomy along with mediastinal lymph node dissection is performed. Gross examination reveals a 12.5 cm yellow-white mass with central cavitation that seems to be invading into the visceral pleura, causing retraction. The tumor is positive for cytokeratin 5/6 and p63 and negative for TTF-1, Napsin A, HMB-45, SOX-10, cytokeratin 7, and cytokeratin 20. Master List of Diagnoses (2658) Large cell carcinoma (1165) Melanoma (1421) Pleomorphic carcinoma (1388) Poorly differentiated adenocarcinoma (5605) Poorly differentiated squamous cell carcinoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following is most distinctive of poorly
differentiated squamous cell carcinoma (SCC)? (6079) Immunoreactivity for p63 and p40 (6080) Immunoreactivity for HMB-45 (2205) Presence of necrosis (5942) Translocations involving chromosome 12 (6081) Tumor cells forming glands
2. Which of the following is true of poorly differentiated lung
SCC? (6082) Distant metastases are not seen. (6083) Prognosis is independent of stage. (6084) Stage for stage, survival rate is significantly better
than for adenocarcinoma. (6085) These tumors show large areas of keratinization. (6086) Tumor cells express TTF-1.
3. Which of the following is most typical of lung
adenocarcinoma? (6087) Common alterations include EGFR, KRAS, and
BRAF (6088) Express p63 and p40 (6089) Grossly well-circumscribed (6090) Least commonly seen in the periphery of the lung (6091) Most are central tumors luminal necrosis
Case # 2019-07 症例 # 2019-07 Site: Testis Clinical Summary A 40-year-old man presents with a painless, palpable testicular mass. Scrotal ultrasonography shows a 3.5 cm homogeneous, hypoechoic intratesticular mass. Serum tumor markers show normal values for alpha fetoprotein (AFP), human chorionic gonadotropin (hCG), and lactate dehydrogenase (LDH). An orchiectomy is performed which reveals a 3.5 cm solid, soft, circumscribed, homogeneous, tan mass confined to the testicular parenchyma. Master List of Diagnoses (2716) Diffuse large B-cell lymphoma, NOS (1994) Embryonal carcinoma (5607) Monophasic choriocarcinoma (2383) Seminoma (2341) Sertoli cell tumor (5608) Spermatocytic tumor (2467) Yolk sac tumor Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Question 1. Which of the following tumors may show extensive
Case # 2019-08 症例 # 2019-08 Site: Retroperitoneum Clinical Summary A 70-year-old woman presents with increasing abdominal girth, discomfort, and bilateral leg swelling over several months. She also reports a weight gain of 60 lbs during this period, early satiety, and decreased appetite. Radiologic studies show a large abdominopelvic/retroperitoneal mass measuring 34.0 cm in greatest dimension and compressing the inferior vena cava. The resected mass weighs 17 kilograms and displays a lobulated yellow cut surface. Master List of Diagnoses (1700) Myxoid liposarcoma (1511) Pleomorphic liposarcoma (5606) Renal angiomyolipoma (2256) Well-differentiated liposarcoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following is most distinctive of well-
(6106) Dedifferentiation results in a distinct change in the cytogenetic profile.
(6107) Distant metastases are common. (6108) Prognosis is independent of anatomic location. (6109) Surgical resection with clear margins is the treatment
of choice. (6110) Tumors occurring in the extremities have higher rate
of recurrence. 3. Which of the following is most typical of pleomorphic
liposarcoma? (6111) Areas of WD-LPS at the periphery (6112) CDK4 gene amplification on chromosome 12 (5945) Hypocellularity with prominent myxoid background
with a rich capillary network (6113) Non-lipogenic component resembling
Case # 2019-09 症例 # 2019-09 Site: Uterine cervix Clinical Summary The patient is a 17-year-old girl who presents with complaints of irregular vaginal bleeding. On clinical exam, she has a gelatinous multi-lobulated lesion protruding from the cervical os. The lesion is removed and submitted as a “cervical polyp” for histopathologic examination. The submitted tissue has a mucoid appearance and measures 19.0 cm in aggregate. Individual tissue fragments have a grape-like appearance. Master List of Diagnoses (2424) Adenosarcoma (1698) Aggressive angiomyxoma (1843) Alveolar rhabdomyosarcoma (1951) Carcinosarcoma (2468) Embryonal rhabdomyosarcoma Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following features best distinguishes
embryonal rhabdomyosarcoma (botryoid subtype) from adenosarcoma? (6115) Absence of staining for CD10, WT1, ER, and PR (6116) Positive staining for desmin and myogenin (6117) Presence of a condensed layer of cells around
glands (6118) Presence of a reproducible cytogenetic translocation (6119) Presence of rhabdomyoblasts
2. Which of the following features best separates embryonal
subtype of rhabdomyosarcoma (ERMS) from the alveolar subtype of rhabdomyosarcoma (ARMS)? (6120) ARMS has an alveolar pattern with fibrovascular
septae. (6121) ARMS has a characteristic gene translocation. (6122) ARMS is a soft tissue-based lesion. (6123) ERMS is more likely to have numerous
rhabdomyoblasts. (6124) ERMS is more pleomorphic than ARMS.
3. Which of the following scenarios is most consistent with
sarcoma botryoides? (6125) A 15-year-old with a pelvic mass and metastases to
the lung (6126) A 25-year-old with a deep soft tissue lesion
surrounding the rectum (6127) A 25-year-old with a polypoid lesion protruding
through the cervical os (6128) A 35-year-old with an endometrial polyp and
abnormal bleeding (6129) A 65-year-old with uterine enlargement and abnormal
Case # 2019-10 症例 # 2019-10 Site: Anterior neck Clinical Summary A 68-year-old woman presents with a rapidly enlarging neck mass of one month’s duration. She also has recent onset dysphagia, dysphonia, and significant weight loss. Clinical examination reveals a firm, fixed mass in the anterior neck, and imaging reveals a large mass centered on the thyroid gland with associated cervical lymphadenopathy. The patient undergoes a surgical resection of the neck mass. Gross examination reveals a firm nodular mass replacing the thyroid and measuring 9.5 x 8.3 x 5.4 cm. Areas of necrosis are present. Master List of Diagnoses (5609) Anaplastic thyroid carcinoma (1620) Malignant melanoma (2048) Medullary thyroid carcinoma (5610) Pleomorphic undifferentiated sarcoma (2272) Poorly differentiated thyroid carcinoma (1600) Riedel thyroiditis Slide Quality: Evaluate the slide for technical quality
1 2 3 4 5 Excellent Satisfactory Unsatisfactory Educational Questions 1. Which of the following immunohistochemical stains is most