Case Of Proximal-type Epithelioid Sarcoma Research Articles

Proximal-type epithelioid sarcoma: A new case report

Epithelioid sarcoma (ES) is a rare high-grade soft tissue sarcoma, first described by Enzinger in 1970, classically presenting as a subcutaneous or deep dermal mass in the distal extremities of adolescents and young adults. In 1997, Guillou et al. described the proximal-type epithelioid sarcoma, which is found mostly in the pelvic, perineal regions, and genital tracts of young to middle-aged adults. Epithelioid sarcoma is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. We present, in this paper, a case of proximal-type epithelioid sarcoma of abdominal wall, with an aggressive clinical course, including distant metastasis and death one month after diagnosis. In this particular case, the location is infrequent, and it had not previously been described.

Ultrasonography findings of proximal-type epithelioid sarcoma of the vulva: A case report.

Proximal-type epithelioid sarcoma (PES) of the vulva is an exceedingly rare malignant soft tissue tumor. We herein present the case of a 41-year-old female patient who presented to our hospital with complaints of a painless mass in the right mons pubis that she had first noticed 3 years prior. Ultrasonographic (US) and color Doppler ultrasonographic (CDUS) examination revealed a solid mass with well-defined, homogeneous hypoechoic structure and quite hypervascular on CDUS. The results of the immunohistochemical examination confirmed the diagnosis of vulvar PES (myxoid variant). The patient was treated with wide local excision and remained recurrence- and metastasis-free at 9 months postoperatively. Although cases of PES in the pelvic region had been previously reported, to the best of our knowledge, the US or CDUS findings of PES of the vulva have not been described to date.

Proximal-type epithelioid sarcoma - Case report

Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.

Proximal-Type Epithelioid Sarcoma of the Scrotum

Proximal-type epithelioid sarcoma is an uncommon but aggressive soft-tissue malignancy involving proximal body parts especially perineum and genitalia of adult patients. This entity is a variant form of the conventional epithelioid sarcoma, which is frequently encountered at extremities of adolescents or young adults. The authors present a case of proximal-type epithelioid sarcoma of the scrotum with comprehensive staging and monitoring with PET/CT.

A case of proximal-type epithelioid sarcoma arising in the base of skull

Epithelioid sarcoma is a rare soft tissue tumour recently shown to display a characteristic loss of INI1 expression. It is subdivided into conventional (distal) and proximal forms, the latter showing large epithelioid carcinoma-like and/or rhabdoid cells. Epithelioid sarcoma rarely arises in the head and neck region, with only seven cases reported in an article by the French Sarcoma Group. In this report, we detail an instance where a proximal-type epithelioid sarcoma affected the skull base with involvement of the frontal lobe of the brain as well as extension into the nasal cavity of a 55-year-old Malay man. The excision specimen revealed a malignant epithelioid tumour forming sheets and lobules. Rhabdoid cells were prominent. The tumour cells showed widespread loss of nuclear expression of INI1, strong diffuse positivity for cytokeratin Cam 5.2 and epithelial membrane antigen, and focal positivity for synaptophysin, chromogranin and vimentin. To the best of our knowledge, this is the first case of a proximal-type epithelioid sarcoma arising from the base of skull and the second case arising from the bone. The histological differential diagnoses of skull base involvement by proximal type epithelioid sarcoma and its pathology are presented.

Proximal-Type Epithelioid Sarcoma in the Groin Presenting as a Diagnostic Dilemma

Epithelioid sarcoma is an uncommon soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in the distal extremities of young adults. Lately, a 'proximal' subtype has been described, which occurs in the pelvic and genital areas of somewhat older individuals and tends to behave more aggressively than the conventional subtype. The correct diagnosis of this subtype is essential, since this tumor can be easily mistaken for other malignant tumors that exhibit epithelioid morphology. We report a case of proximal-type epithelioid sarcoma that presented as an inguinal mass in a 47-year-old man. Histologically, the tumor consisted of diffuse sheets of epithelioid cells with scattered rhabdoid morphology. By immunohistochemistry, the neoplastic cells expressed cytokeratin, epithelial membrane antigen, vimentin, CD34, CD99 and showed complete loss of nuclear INI1 protein expression. Fluorescence in situ hybridization was considered borderline for 22q deletion. We present this case to emphasize the importance of diagnosing this uncommon tumor and the role of INI1 immunohistochemistry in establishing the diagnosis.

Clinicopathological study of 5 cases of proximal-type epithelioid sarcoma

To investigate the clinicopathological characteristics, diagnosis and differential diagnoses of proximal-type epithelioid sarcoma (PES). Five cases of PES were retrieved from pathology files. Clinical, pathologic and immunohistochemical features of the tumors were reviewed. One patient was female and 4 were male. Ages of the patients ranged from 19 to 46 years. The sites of the tumor involvement were vulvar (2 cases), hypogastric zone (1 case), anterosuperior iliac spine (1 case) and buttock (1 case). Clinically, the tumor masses were painless and progressive solitary nodules. Microscopically, the tumor cell growth was infiltrative in nature, nodular in appearance with degenerative and necrotic cells at the central areas. The tumors consisted of relatively uniform epithelioid cells with round or oval nuclei and eosinophilic cytoplasm. Immunohistochemically, the tumor cells were positive for vimentin (5/5), CK (4/5), EMA (4/5), beta-catenin (3/5), CD34 (3/5), and S-100 protein (1/5), but were negative for SMA, MyoD1, Desmin, HMB-45, CK7 and CK20. Definitive diagnosis of PES relies on its histopathological characteristics in conjunction with appropriate immunohistochemical findings.

Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India

Epithelioid sarcoma (ES) is an uncommon sarcoma. Lately, its variants, including proximal-type ES, have been recognized. The present study highlights clinicopathological features of 26 (65%) conventional and 14 (35%) cases of proximal-type ES. Thirty-eight percent of cases were seen in 21-30-year age group, including 77.5% cases in men. Extremities were the commonest sites in both the subtypes. Histologically, conventional-type ES displayed nodular tumor aggregates with necrosis, while proximal-type showed solid arrangement of large, "rhabdoid-like" cells. More cases (64.2%) of the proximal type were of grade 3. A range of differential diagnoses was considered. Most important immunohistochemical markers were vimentin, epithelial membrane antigen, cytokeratin, CD34, and desmin. Maximum (72.5%) cases were treated surgically. Recurrences and metastasis were observed more in the proximal type. The 7-year disease-free survival was 19.4% in the conventional and nil in the proximal subtype (p = 0.06). The overall survival rate was also lower in the proximal (31.3%) than conventional type (90.2%; p < 0.001). Other unfavorable parameters were deeper location, larger size, and higher tumor stage. This unusual sarcoma, with characteristic growth patterns, merits a proper histological evaluation, as it has many mimics. Proximal-type ES is rather a morphological subtype, associated with an aggressive course.

Proximal-Type Epithelioid Sarcoma With Elevated Serum CA 125: Report of a Case With CA 125 Immunoreactivity

Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.

Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity.

Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.

Proximal-Type Epithelioid Sarcoma: A Clinicopathologic Study of 20 Cases

We studied the clinicopathologic and immunohistochemical features of 20 cases of proximal-type epithelioid sarcoma to identify prognostic factors. The 20 patients ranged in age from 13 to 80 years (mean, 40 y); 12 patients were male and 8 were female. The tumors presented as deep soft-tissue or subcutaneous masses on the inguinal region in five, the thigh in four, the vulva in three, the axilla in three, and one each in the flank, chest wall, back, hip and perineum. The tumors ranged from 2 to 16 cm at their greatest diameter (mean: 7.8 cm). Histologically, 12 tumors (60%) were classified as the large-cell subtype, characterized by sheets of large cells with prominent nucleoli resembling poorly differentiated carcinoma, and a frequent rhabdoid phenotype, six (30%) were classified as the conventional subtype, and two (10%) as the angiomatoid subtype. The numbers of tumors exhibiting immunoreactivity for various markers were: vimentin (20 cytokeratin (20 [100%]); epithelial membrane antigen (17 [85%]); CD34 (9 [45%]); CD99 (5 [25%]); muscle markers, either desmin or α-smooth muscle actin (3 [15%]), other markers such as S-100 protein, neurofilament, neuron-specific enolase, synaptophysin and CD56 (12 [60%]); and p53 (16 [80%]). Fourteen lesions (70%) exhibited an MIB-1 index of 30% or more and, by a system of histologic grading using the MIB-1 score, 16 tumors (80%) were classified as high-grade (Grade 3). Thirteen patients (65%) developed local recurrence and 15 (75%) had metastases, primarily to the lymph nodes. At the last follow-up, 13 patients (65%) had died of their disease. A large tumor size and early metastasis were independently associated with a poor outcome. We conclude that proximal-type epithelioid sarcomas are rare, undifferentiated soft-tissue sarcomas of adults, with epithelioid features and a frequent rhabdoid phenotype. These tumors, when arising in proximal locations, have a much worse prognosis than those arising in distal locations.