Lymphangioma is a rare proliferation of the lymphatic system which is classified as either lymphangioma circumscription or carvenous lymphangioma. The involvement of the vulva is very rare and only a small number of case reports have been made on carvenous lymphangioma of the vulva. We herein report a case of 20-year-old unmarried girl presented with gradually expanding and painless tumor of the left labium majus. The mass was removed surgically and pathology confirmed as carvenous lymphangioma, with no recurrences to date.
Female ; Humans ; Lymphangioma* ; Lymphatic System ; Pathology ; Recurrence ; Single Person ; Vulva* ; Vulvar Neoplasms ; Young Adult

Aged ; Female ; Humans ; Leukocyte Common Antigens ; metabolism ; Peroxidase ; metabolism ; Sarcoma, Myeloid ; metabolism ; pathology ; Vulva ; metabolism ; pathology ; Vulvar Neoplasms ; metabolism ; pathology

Behcet's disease is a multi-systemic vasculitis and characterized by systemic organ involvement. Although the gastrointestinal and systemic features of Behcet's disease and inflammatory bowel disease overlap to a considerable extent, they are generally viewed as two distinct diseases. A 39-yr-old female was diagnosed as having Behcet's disease. She was admitted to our hospital because of oral and genital ulcer, lower abdominal pain, and frequent diarrhea. Colonosopy showed diffuse involvement of multiple longitudinal ulcers with inflammatory pseudopolyps with a cobblestone appearance and ano-rectal fistula was suspected. These findings are extremely rare in Behcet's disease. However, there were no granulomas, the hallmark of Crohn's colitis. Microscopically, perivasculitis and multiple lymph follicles compatible with Behcet's disease were seen. Although being rarely encountered, multiple longitudinal ulcers, cobblestone appearance, and ano-rectal fistula can develop in Behcet's disease, as in Crohn's colitis. Therefore, Behcet's disease and Crohn's disease may be closely related and part of a spectrum of disease.
Adult ; Behcet Syndrome/diagnosis/*therapy ; Colitis ; Colonoscopy ; Crohn Disease/diagnosis/*therapy ; Female ; Humans ; Inflammation ; Inflammatory Bowel Diseases/pathology ; Intestinal Diseases/diagnosis/*therapy ; Perineum/pathology ; Ulcer ; Vulva/pathology

BACKGROUND: Extramammary Paget's disease (EMPD) is a relatively rare cutaneous disorder. There are a few studies regarding the clinical features and prognostic factors of EMPD in Korean patients. OBJECTIVE: The aim of this study was to identify in detail the clinical findings and prognostic factors of EMPD in Korean patients. METHODS: A total of 19 patients with confirmed EMPD at our institution were included from January 2005 to December 2014. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS: Patients' ages ranged from 50 to 87 years (median, 64.5 years), and incidence of EMPD was higher in men than in women (17:2, male:female). The most common predilection site was the penoscrotal area in men and the vulva in women. All patients with EMPD had no underlying malignancy. Four of 19 patients treated with wide excision had local recurrence during the follow-up period. Two patients with deep dermal invasion died due to multiple metastases. CONCLUSION: EMPD in Korea is characterized by its male predominance and penoscrotal location. Disease recurrence is common regardless of the surgical margin. Its prognosis could be associated with depth of invasion and lymph node metastasis.
Female ; Follow-Up Studies ; Humans ; Incidence ; Korea ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Paget Disease, Extramammary* ; Pathology ; Prognosis ; Recurrence ; Retrospective Studies ; Vulva

OBJECTIVETo study the clinicopathological features, immnophenotype and differential diagnosis of prepubertal-type vulva fibroma (PVF).

METHODClinical, radiological and pathological features of 2 cases of PVF were analyzed along with a literature review.

RESULTSBoth patients were females of 8.5 and 54 years of age, presented with recurrent unilateral mass at the labium major respectively. Grossly, cut surface of the tumor appeared as the gray fibrous tissue without any definited lump detected. Histologically, the ill-circumscribed lesion located predominantly in the deep dermis with an extension into the subcutaneous tissue. They had a low cell density consisting of scattered spindle shaped fibroblast-like cells and a large amount of collagen fiber matrix, nuclear atypia not seen and mitotic figures scanty. The tumor cells extended downward under the epithelium and infiltrated between the fat tissue, nerve fibers as well as the capillaries making a lesion looked somewhat like a harmatoma. Immunohistochemically, the spindle cells were uniformly positive for vimentin, weakly positive for CD34, and negative for alpha-smooth muscle actin (SMA), muscle specific actin (HHF35), desmin, h-caldesmon, CD99, S-100 protein, bcl-2, beta-catenin, estrogen and progesterone receptors.

CONCLUSIONSPVF is a benign mesenchymal lesion with a predilection of involving the vulva of prepubertal girls or adults in rare cases. PVF may represent an overgrowth of normal stromal tissue of vulva. Approximately one third of the tumors develop local recurrence due to incomplete excision, however, there is also occasionally spontaneous regression.

Antigens, CD34 ; metabolism ; Child ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Humans ; Middle Aged ; Myxoma ; pathology ; Neoplasm Recurrence, Local ; Vimentin ; metabolism ; Vulva ; pathology ; surgery ; Vulvar Neoplasms ; metabolism ; pathology ; surgery

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

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