A case of primary non-Hodgkin's malignant lymphoma of the vulva which occurred in a 68-year-old woman is presented. Non-Hodgkin's malignant lymphoma is infrequently involved in the female genital tract. Moreover, primary vulvar involvement of this tumor is very rare. To date only 6 cases have been reported in the literature. To our knowledge this is the first reported case of a non-Hodgkin's malignant lymphoma of the vulva in Korea.
Aged ; Female ; Humans ; Lymphoma, Non-Hodgkin/*pathology/therapy ; Vulvar Neoplasms/*pathology/therapy

The phrase "locally advanced carcinoma of the vulva" has often been mentioned in the literature, though not accurately defined, or even leading to the interpretation overlapping. Grounded on cervical cancer experience, we are able to state that designing a tailored primary strategy based on clinically measurable adverse prognostic factors represents the cornerstone of therapy. This fact urged us to rethink about the real usefulness of the concept of locally advanced squamous cell carcinoma of the vulva. We will refer to this concept as a clinical entity emerging from a rigorous workup which is a valuable guiding tool in the context of a thorough debate about the best primary treatment approach to be used. Furthermore, bulky tumors of the vulva have been associated with a worse prognosis on several occasions. Some authors have questioned the fact that tumor size has not been considered in the staging system. Finally, a standardized definition will help us compare the results obtained, which is extremely necessary given the worldwide low prevalence of this disease.
Carcinoma, Squamous Cell/*pathology/therapy ; Combined Modality Therapy ; Evidence-Based Medicine/methods ; Female ; Humans ; Neoplasm Staging ; Prognosis ; *Terminology as Topic ; Vulvar Neoplasms/*pathology/therapy

Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Infant ; Ki-67 Antigen ; metabolism ; Mesenchymoma ; drug therapy ; metabolism ; pathology ; surgery ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; Rhabdomyosarcoma, Embryonal ; drug therapy ; metabolism ; pathology ; surgery ; Teratoma ; metabolism ; pathology ; Vulvar Neoplasms ; drug therapy ; metabolism ; pathology ; surgery

OBJECTIVEVulvar epithelioid sarcoma is a rare, undifferentiated soft-tissue sarcoma, with a high rate of local relapse, regional nodal spread and distant metastases. The aim of this study was to investigate the clinical features, diagnosis, treatment and prognosis of this malignancy.

METHODSWe studied the clinicopathologic features of 20 cases of vulvar epithelioid sarcoma, of which 4 cases were admitted to our hospital from 1999 to 2009. All of the patients received radical local excision with inguinofemoral lymphadenectomy. Seven patients were treated without adjuvant therapy. Seven patients received postoperative radiotherapy only and three underwent chemotherapy. Chemotherapy plus radiotherapy were given postoperatively in three.

RESULTSThe patients ranged in age from 23 to 80 years (median: 36 y). The tumors ranged from 1 to 10 cm in their greatest diameter (median: 5.1 cm). All cases showed immunoreactivity for both vimentin and cytokeratin. Follow-up information on all 20 patients was available, and covered periods ranging from 3 to 104 months.11 patients were alive with no evidence of disease. 2 patients developed lymph node metastases but alive. 7 patients had died of the disease. Survival of the early stage (I-II) patients was significantly longer than those in the advanced stage (III-IV) (median, 21 vs. 6 months, P < 0.01). There was no significant difference between survival of patients with or without inguinofemoral lymphadenectomy (median, 11.5 vs. 6 months, P = 0.086).

CONCLUSIONSBecause of the relatively frequent misdiagnosis, a differential diagnosis combined with immunohistochemistry is needed to determine an early and accurate diagnosis. The tumor markers exhibiting immunoreactivity includ vimentin, epithelial membrane antigen (EMA) and cytokeratin (CK). Radical local excision with adequate margin (at least 2 cm) and bilateral inguinofemoral lymphadenectomy is effective for the treatment of vulvar epithelioid sarcoma. The role of adjuvant therapy, chemotherapy and radiation remains unclear but merits consideration.

Adult ; Aged ; Aged, 80 and over ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Humans ; Keratins ; metabolism ; Lymph Node Excision ; Lymphatic Metastasis ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Staging ; Radiotherapy, Adjuvant ; Retrospective Studies ; Sarcoma ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Survival Rate ; Vimentin ; metabolism ; Vulva ; surgery ; Vulvar Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Young Adult

OBJECTIVETo evaluate the clinicopathological characteristics and treatment of adenoid cystic carcinoma of the Bartholin gland.

METHODSThe clinicopathological data of six patients with adenoid cystic carcinoma of the Bartholin gland were retrospectively analyzed. The median age was 40.8 years (range 30 to 54 years). Surgery was the primary treatment. Simple vulvar tumor resection was performed in 1 patient. Four cases underwent radical vulvectomy with bilateral inguinal lymph node dissection and 1 case underwent wide local excision of the vulva with bilateral inguinal lymph node biopsy. Two cases with high risk factors received postoperative radiotherapy.

RESULTSAll patients had definite pathological diagnosis. Cribriform arrangement of tubules and gland-like elements and infiltration of perineural spaces were two main microscopic features of this type of tumor. The pathological examination after surgery revealed that two patients had positive surgical margins, one had negative margin, 1 adjacent to the tumor and 1 unknown; 5 cases had negative inguinal lymph nodes and 1 unknown. All the 6 patients were followed-up. Recurrence developed in 4 cases including 3 with both local recurrence and lung metastasis, and one had lung metastasis only. One patient died of lung metastasis and her total survival period was 135 months. The other 3 recurrent patients survived with tumor and the total survival period was 241, 128 and 103 months, respectively. Two cases without recurrence survived 8 and 121 months, respectively.

CONCLUSIONAdenoid cystic carcinoma of the Bartholin gland is a slow growing but locally very aggressive neoplasm with a high capacity for local recurrence and lung metastasis. Surgery is the most common and useful treatment. Radiation is a choice of treatment for patients with high risk factors after surgery such as positive surgical margin, deep local invasion and infiltration of perineural spaces or for recurrent patients without opportunity of excision.

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bartholin's Glands ; pathology ; surgery ; Carcinoma, Adenoid Cystic ; pathology ; radiotherapy ; secondary ; surgery ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms ; secondary ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Middle Aged ; Neoplasm Recurrence, Local ; drug therapy ; radiotherapy ; surgery ; Radiotherapy, High-Energy ; Retrospective Studies ; Survival Rate ; Vulva ; surgery ; Vulvar Neoplasms ; pathology ; radiotherapy ; surgery