Pregnancy ; Female ; Humans ; Ovary/pathology* ; Gestational Trophoblastic Disease/pathology* ; Trophoblastic Neoplasms/pathology* ; Uterine Neoplasms/pathology* ; Epithelioid Cells/pathology*

Lupus miliaris disseminatus faciei (LMDF) is a rare granulomatous skin disease mainly affecting the central area of the face. A variety of treatments are reportedly of some benefit; however, controlled studies to establish the best treatment are lacking. Here, we report the cases of a 33-year-old man who presented with multiple, various-sized, erythematous maculopapules on the face and a 19-year-old man who presented multiple, reddish papuloplaques distributed on the face. Histopathological examinations of the two cases revealed large clear-boundary epithelioid cell granulomas with central necrosis surrounded by lymphocytic infiltration. Based on the clinical and histological findings, diagnoses of LMDF were made. As oral tetracycline and antimalarials were not fully effective in our cases, oral mini-pulse steroid therapy (dexamethasone, 5 mg bid for 2 days per week) was initiated. After several months, the eruption significantly improved, and most lesions were resolved. Here, we report two cases of LMDF successfully treated with oral mini-pulse steroid therapy.
Adult ; Antimalarials ; Diagnosis ; Epithelioid Cells ; Granuloma ; Humans ; Necrosis ; Skin Diseases ; Tetracycline ; Young Adult

Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.
Actins ; Adolescent ; Breast ; Carcinoma, Renal Cell ; Epithelioid Cells ; Female ; Humans ; MART-1 Antigen ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms ; Sarcoma, Alveolar Soft Part

Solitary fibrous tumor (SFT) is a rare, soft tissue neoplasm that rarely presents in breast tissue, with only 27 previously reported cases. To our knowledge, only one case of malignant SFT has been reported in the English literature. A 75-year-old Caucasian woman presented to our institution with a 3-month history of a palpable left breast mass. No other symptoms, including nipple discharge or skin changes, were noted. She underwent 3 previous biopsies for right breast masses, all of which were benign, with no evidence of spindle cell neoplasm, atypical hyperplasia, or malignancy. Microscopic examination of the mass demonstrated a classic area of SFT with areas of high-grade anaplastic component. In these areas, the tumor showed atypical epithelioid cells arranged in hypercellular sheets with diminished branching vasculature, nuclear pleomorphism, and increased mitotic count (up to 9/10 high-power fields). This case represents the second case of malignant SFT in the breast.
Aged ; Biopsy ; Breast ; Epithelioid Cells ; Female ; Hemangiopericytoma ; Humans ; Hyperplasia ; Nipples ; Skin ; Soft Tissue Neoplasms ; Solitary Fibrous Tumors

Primary malignant melanoma of the breast (PMMB) is a rare tumor with only a few case reports available in the literature. We report two cases of PMMB, one derived from the breast parenchyma and the other from the breast skin. The first case consisted of atypical epithelioid cells without overt melanocytic differentiation like melanin pigments. The tumor cells showed diffuse positivity for S100 protein, tyrosinase, and BRAF V600E. However, the tumor cells were negative for cytokeratin, epithelial membrane antigen, and HMB-45. The second case showed atypical melanocytic proliferation with heavy melanin pigmentation. The tumor cells were positive for S100 protein, HMB-45, tyrosinase, and BRAF V600E. These two cases represent two distinct presentations of PMMB in terms of skin involvement, melanin pigmentation, and HMB-45 positivity. Although PMMB is very rare, the possibility of this entity should be considered in malignant epithelioid neoplasms in the breast parenchyma.
Breast ; Epithelioid Cells ; Keratins ; Melanins ; Melanoma ; Monophenol Monooxygenase ; Mucin-1 ; Pigmentation ; Skin

No abstract available.
Epithelioid Cells* ; Granuloma, Pyogenic* ; Histiocytoma* ; Histiocytoma, Benign Fibrous

Epithelioid hemangioendothelioma is a rare vascular tumor with intermediate malignity and metastasis risk. It presents epithelioid cells with intracytoplasmic vacuoles and low mitotic activity. Its vascular nature can be confirmed by immunohistochemical studies (vimentin, CD31, CD34, and factor VIII). It is extremely rare in the nasal cavity, with only one case reported on the middle turbinate in Korea. The authors present a case of epithelioid hemangioendothelioma on the choana with a size of 2mm, which easily coult have been misdiagnosed as a blood clot.
Epistaxis ; Epithelioid Cells ; Hemangioendothelioma, Epithelioid* ; Korea ; Nasal Cavity ; Neoplasm Metastasis ; Turbinates ; Vacuoles

Epithelioid angiomyolipoma (EAML) of liver is a rare neoplasm. Hepatic EAML is often misdiagnosed as other neoplasms such as hepatocellular carcinoma due to non-specific clinical and radiologic features. The morphologic features under microscope and immunohistochemistry staining profile are important in the diagnosis EAML. Here, we report a case of 52-year-old man who found 1.2 cm mass in liver by routine checkup. On the impression of hepatocellular carcinoma, lateral sectionectomy of the liver was done. Microscopically, the tumor is composed of predominant epithelioid cells with vascular component and foamy cells. These cells were positive for HMB45, MelanA, and smooth muscle actin and negative for epithelial membrane antigen. The final diagnosis was hepatic EAML.
Actins ; Angiomyolipoma* ; Carcinoma, Hepatocellular ; Diagnosis ; Epithelioid Cells ; Humans ; Immunohistochemistry ; Liver* ; MART-1 Antigen ; Middle Aged ; Mucin-1 ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms

Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45) and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA).
Actins ; Adipose Tissue ; Blood Vessels ; Carcinoma, Hepatocellular ; Diagnostic Imaging ; Epithelioid Cells* ; Female ; Hematopoiesis, Extramedullary ; Hepatectomy ; Hepatitis C, Chronic ; Hepatocytes ; Humans ; Kidney Failure, Chronic ; Liver ; MART-1 Antigen ; Melanoma ; Middle Aged ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms

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.
Aged ; Diagnosis, Differential ; Epithelioid Cells* ; Esophagogastric Junction ; Female ; Gastrointestinal Stromal Tumors ; Humans ; MART-1 Antigen ; Middle Aged ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms ; Stomach Neoplasms ; Stomach*