Myounghwan Kim

Proximal-type epithelioid sarcoma of the vulva with INI1 diagnostic utility.

Proximal epithelioid sarcoma (PES) is an extremely uncommon neoplasm of the vulva with an aggressive behavior. Recently, these authors experienced a case of proximal-type ES in a 41-year-old woman who was admitted for a rapidly growing mass in the right mons pubis. An about-1-cm-sized mass was initially noticed one and a half years earlier. The excised mass, however, was 8 cm in greatest dimension and was relatively well circumscribed. The cut surface was trabeculated, with multifocal hemorrhages and necroses. Microscopically, the tumor consisted of epithelioid rhabdoid cells with vesicular nuclei, large prominent nucleoli, and cytoplasmic eosinophilic globules comparted by thin, fibrous septae. The main differential diagnoses included PES, other sarcomas with epithelioid cells, malignant melanoma, and sarcomatoid carcinoma. The tumor cells were diffusely positive for vimentin and EMA; focally positive for cytokeratin; and negative for CK5/6, CD34, S-100 protein, desmin, and myogenin. INI1 (hSNF5/SMARCB1, a member of the SW1/SNF chromatin remodeling complex located on chromosome 22q11.2) staining clearly showed loss of expression in the tumor cells. Recent studies reported that some ESs also showed INI1 inactivation, as characteristically seen in malignant rhabdoid tumors of infancy. Reported herein is the diagnostic utility of INI-1 on PES and the possible relationship between PES and malignant rhabdoid tumor of the soft tissue, besides a collective review of the reported cases of PES of the vulva and of the current case.

Carcinosarcoma of the uterine cervix arising from Müllerian ducts

Carcinosarcomas of the uterine cervix are extremely rare. Cervical carcinosarcoma can be characterized by having two different origins: the Müllerian ducts and the mesonephric duct remnants. A 53-year-old Korean woman was admitted to the hospital because of pelvic mass detected on computed tomography scan done at private clinic. A Radical hysterectomy with bilateral salpingooophorectomy and pelvic lymphadenectomy was carried out upon a diagnosis of stage IB2 cervical sarcoma. Immunohistochemically, the epithelial component was positive for pancytokeratin and estrogen receptor, but negative for CD 10 and carletinin. The mesenchymal component was positive for vimentin. The histopathologic diagnosis was a carcinosarcoma of the uterine cervix arising from Müllerian ducts. She underwent chemotherapy. She developed systemic recurrence seven months after operation and died of disease. The origin of cervical carcinosarcoma needs to be verified and immunohistochemical studies using mesonephric marker (CD 10, carletinin, and estrogen receptor) is helpful.

A case of adhesive small bowel obstruction with pelvic inflammatory disease due to Chlamydia trachomatis

17세 여자가 골반염증성질환으로 입원하여 항생제 치료를 시작하였다. 증상 호전 중 입원 5일째 갑자기 오심과 구토 증상이 시작되었다. 보존적 처치에도 불구하고 증상이 심해져 7일째 시행한 단순복부촬영과 복부골반 전산화단층촬영에서 허혈성 소장폐쇄 소견이 보였다. 응급개복술을 시행하였고 소장 장간막 부위에 그물막이 붙어 유착띠가 형성되어 있었고 20 cm 정도의 소장이 끼어 있었다. 부분 그물막절제술, 유착띠박리술, 부수적으로 충수돌기절제술을 시행하였다. 수술 후 증상은 완전히 소실되었으며 11개월간 외래 추적 관찰에서도 특이사항은 없었다. 이 증례는 골반염과 동반된 오심 구토 증상이 지속될 경우 골반염증질환의 드문 합병증인 장폐쇄를 의심하여야 하겠고, 정확한 진단과 의사결정을 위해 단순복부촬영뿐만 아니라 복부골반 전산화단층촬영의 유용성을 보여준다. 저자들은 Chlamydia trachomatis 감염에 의한 골반염성 질환 환자에서 발생한 유착성 소장폐쇄 1예를 경험하였기에 이에 간단한 문헌 고찰을 함께 보고하는 바이다.