Journal of Pathology and Translational Medicine

Case Studies

Perivascular Epithelioid Cell Tumor in the Stomach: Sun Ah Shin, Jiwoon Choi, Kyung Chul Moon, Woo Ho Kim; J Pathol Transl Med. 2017;51(4):428-432. Published online April 4, 2017; DOI: https://doi.org/10.4132/jptm.2016.09.16

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Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST), and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.

Citations

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Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review
Hsiu-Chung Cheng, Chia-Yu Kuo, Ching-Wen Huang, Hsiang-Hung Shih, Chih-Hung Lin, Jaw-Yuan Wang
Journal of International Medical Research.2021; 49(9): 030006052110415. CrossRef
Gastric Perivascular Epithelioid Cell Tumor (PEComa)
Jinghong Xu, Yu Yan, Xueping Xiang, Peter Jiang, Xiangrong Hu, Wenjun Yang
American Journal of Clinical Pathology.2019; 152(2): 221. CrossRef
Robotic wedge resection of a rare gastric perivascular epithelioid cell tumor: A case report
Alessandra Marano, Francesca Maione, Yanghee Woo, Luca Pellegrino, Paolo Geretto, Diego Sasia, Mirella Fortunato, Giulio Fraternali Orcioni, Roberto Priotto, Renato Fasoli, Felice Borghi
World Journal of Clinical Cases.2019; 7(23): 4011. CrossRef

Unilateral Pigmented Extramammary Paget's Disease of the Axilla Associated with a Benign Mole: A Case Study and a Review of Literature: Aleem Ladak, Maria Bramley, Sami Titi; Korean J Pathol. 2014;48(4):292-296. Published online August 26, 2014; DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.4.292

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Abstract PDF

Pigmented extramammary Paget's disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget's disease affecting skin that is rich in apocrine sweat glands such as the axilla, perianal region and vulva. It most commonly occurs in postmenopausal women and presents as a superficial pigmented scaly macule, mimicking a melanocytic lesion. The histological presentation is adenocarcinoma in situ with an increased number of melanocytes scattered between the Paget's cells. Therefore, PEMPD may be misdiagnosed as a melanocytic tumour both clinically and histologically. The tumour cells are usually positive for cytokeratin 7, epithelial membrane antigen, Cam 5.2, HER2, and mucicarmine stain while S100 and human melanoma black-45 highlight the processes of reactive dendritic cells. The association between Paget's cells and intratumoural reactive melanocytes is still unclear. We report our first case of PEMPD associated with an intradermal naevus involving the axilla in a 63-year-old woman.

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Atypical cells on reflectance confocal microscopy may not represent melanoma: A case of axillary pigmented extramammary Paget disease
Nadiya Chuchvara, Catherine Reilly, Attiya Haroon, Cindy Wassef, Amin Maghari, Babar Rao
Journal of Cutaneous Pathology.2020; 47(12): 1170. CrossRef
“Pigmented Extramammary Paget Disease”—A Potential Mimicker of Malignant Melanoma and a Pitfall in Diagnosis: A Case Report and Review of the Literature
Katrin Kiavash, Steve Kim, Andrew D. Thompson
The American Journal of Dermatopathology.2019; 41(1): 45. CrossRef
Vulvar cancer: a review for dermatologists
Anastasiya Atanasova Chokoeva, Georgi Tchernev, Elena Castelli, Elisabetta Orlando, Shyam B. Verma, Markus Grebe, Uwe Wollina
Wiener Medizinische Wochenschrift.2015; 165(7-8): 164. CrossRef

Case Report

Pigmented(melanotic) Schwannoma of the Cervical Spinal Canal: A case report.: Hyunee Yim, Jai Hyang Go, Chang Soo Ahn, Sun Woon Hong, Woo Hee Jung; Korean J Pathol. 1995;29(2):256-262.

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Abstract PDF: Pigmented(melanotic) schwannoma is a very rare variant of schwannoma that characteristically has massive cytoplasmic melanin. Since it was described in 1946 by Bjorneboe, about 44 cases have been reported in the English literature. It has a relatively benign clinical course, but the cases arising in the cranial nerve and sympathetic chain show aggressive behavior with malignant potential. We herein report a typical case of pigmented schwannoma with light microscopic findings. The results of immunohistochemical and electronmicroscopic study are also presented. The patient was a 30 year-old Korean male who had a mass in his posterior neck for 10 years that recently.began to induce neurologic manifestations. The tumor, which was mainly in the extramedullary intradural space of the cervical canal extending to the extradural space and soft tissues of the neck, was relatively well defined and composed of black solid tissue. Microscopic, densely pigmented spindle cells forming fascicles, nuclear palisading, whorling and polygonal cells with vacuolated or clear cytoplasm were characteristic. Immunohistochemically, the tumor cells were positive for vimentin, S-100 protein, and HMB-45. Electron microscopic study revealed that the tumor cells have interdigitating cytoplasmic processes containing varying stages of melanosomes with a very focal basal lamina and Luse body like collagen bundles.

Original Article

Immunohistochemical Evaluation of HMB-45 and S-100 Protein in Melanocytic Tumors.: Chang Soo Park, Hwan Kim, Hyang Mi Ko, Kyung Soo Kim, Ji Shin Lee; Korean J Pathol. 1995;29(2):189-196.

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Abstract PDF: Immunohistochemical staining on paraffin sections for S-100 protein improved diagnostic accuracy for melanocytic tumor. But specificity of S-100 protein in the diagnosis of melanocytic tumor is very low, because S-100 protein was also expressed in neurogenic tumor and salivary gland tumor. To investigate a specific tumor marker for the malignant melanoma, immunohistochemical staining for HMB-45 and S-100 protein was performed on the paraffin sections of 25 cases of malignant melanoma and 46 cases of nevi. Positive reaction for HMB-45 and S-100 protein was diffusely identified in the cytoplasm of tumor cells. Positive ratio for HMB-45 was 100% in malignant melanoma, 92% in junctional component of compound nevus and 0% in intradermal nevus. Positive ratio for S-100 protein was 92% in malignant melanoma, 100% in compound nevus and 100% in intradermal nevus. The sensitivity and specificity for HMB-45 in malignant melanoma were 100%, but those for S-100 protein were 92% in sensitivity and 86.7% in specificity. These results indicate that HMB-45 has a high sensitivity and specificity for malignant melanoma cells and it can be quite useful for the histopathological diagnosis of malignant melanoma.

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