Indian Journal of Pathology and Microbiology

: 2019  |  Volume : 62  |  Issue : 4  |  Page : 647--649

Catechism (Quiz 6)

Bharat Rekhi 
 Department of Surgical Pathology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India

Correspondence Address:
Bharat Rekhi
Room Number 818, Department of Surgical Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Dr E.B. Road, Parel, Mumbai - 400 012, Maharashtra

How to cite this article:
Rekhi B. Catechism (Quiz 6).Indian J Pathol Microbiol 2019;62:647-649

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Rekhi B. Catechism (Quiz 6). Indian J Pathol Microbiol [serial online] 2019 [cited 2023 Sep 27 ];62:647-649
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A 16-year-old girl presented with a swelling in the medial aspect of her knee of 1-month duration. During clinical examination, a large soft-tissue mass was identified over the medial aspect of her left knee.

Computed tomogram (CT) showed a well-defined hypodense mass in the medial aspect of her right distal thigh, measuring 7 cm × 5.1 cm in maximum axial dimensions, displacing the vastus intermedius and extending inferiorly into the patellofemoral compartment of the knee joint. She underwent a biopsy, followed by complete excision.

Gross appearance of the resected specimen and microscopic images from the resected tumor have been displayed [Figure 1] and [Figure 2].{Figure 1}{Figure 2}{Figure 3}


Q1. What is the diagnosis?

Q2. What other name is assigned to this tumor after the name of the pathologist who described it for the first time?

Q3. Which specific immunohistochemical marker for this tumor is displayed in figure 3?

Q4: This tumor shares a morphological continuum with which other tumor(s).

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Conflicts of interest

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 Answer of Catechism (Quiz 5)

Diagnosis: clear cell carcinoma of ovaryLikely specific immunohistochemical marker: Napsin AOther sensitive and specific marker: Hepatocyte nuclear factor 1 beta and IMP3Which clinical history should be checked: Endometriosis.


Microscopic examination of sections revealed a tumor composed of solid-cystic components, including cells with vacuolated cytoplasm and prominent nuclei and nucleoli, arranged in solid and papillary growth patterns, including 'hobnail-like' arrangement of tumor cells around hyalinized stroma [Figure 1].{Figure 4}

By immunohistochemistry, tumor cells were diffusely positive for cytokeratin (CK)7 and Napsin A (granular cytoplasmic staining) [Figure 2], focally positive for glypican 3, while negative for WT1 and ER. In addition, tumor cells showed focal immunostaining for WT1 (Wild type).{Figure 5}

Clear cell carcinoma of the ovary is one of the relatively uncommon subtypes of an epithelial ovarian cancer (EOC). In view of its overlapping histopathological features with other ovarian tumors, such as a yolk sac tumor and various other EOCs, it can be misdiagnosed, as occurred in this case, which was diagnosed as a high grade serous adenocarcinoma (HGSC), elsewhere.[1],[2]

Careful assessment of histopathological features, such as vacuolated cells with prominent nuclei arranged in a 'hobnail-like' arrangement around hyalinised papillary cores, as noted in the present case, are useful morphological clues. Furthermore the diagnosis can be confirmed by positive expression of certain sensitive and specific immunohistochemical markers, such as Napsin A, Hepatocyte nuclear factor-1beta (HNF-1beta) and IMP3 and negative expression of WT1.[2],[3],[4] A clinical history of co-existing endometriosis should be checked in such cases. An exact diagnosis has significant treatment implications, as CCC ovary is relatively chemo refractory, in contrast to a HGSC.[5] After completing two cycles of neoadjuvant chemotherapy, there was no significant clinical response in the present case.



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2Rekhi B, Deodhar KK, Menon S, Maheshwari A, Bajpai J, Ghosh J, et al. Napsin A and WT 1 are useful immunohistochemical markers for differentiating clear cell carcinoma ovary from high-grade serous carcinoma. APMIS. 2018;126:45-55.
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4Lu L, Wang S, Zhu Q, Qu Y, Gu W, Ning Y, Chen X, Wang Y. The expression of IMP3 in 366 cases with ovarian carcinoma of high grade serous, endometrioidand clear cell subtypes. Pathol Res Pract 2018; 214:1087-1094.
5Goff BA, Sainz de la Cuesta R, Muntz HG, Goff BA, Sainz de la Cuesta R, Muntz HG. Clear cell carcinoma of the ovary: A distinct histologic type with poor prognosis and resistance to platinum-based chemotherapy in stage III disease. Gynecol Oncol 1996;60:412-417.