OBJECTIVE: To identify suitable diagnostic tools and evaluate the efficacy of sentinel lymph node (SLN) biopsy for inguinal lymph node metastasis in vulvar cancer. METHODS: Data from 41 patients with vulvar cancer were evaluated retrospectively, including magnetic resonance imaging (MRI) measurements, SLN biopsy status, groin lymph node metastasis, and prognosis. RESULTS: SLN biopsy was conducted in 12 patients who had stage I to III disease. Groin lymphadenectomy was omitted in five of the nine patients with negative SLNs. All SLN-negative patients who did not undergo groin lymphadenectomy showed no evidence of disease after treatment. On MRI, the long and short diameters of the inguinal node were significantly longer in metastasis-positive cases, compared with negative cases, in 25 patients whose nodes were evaluated pathologically (long diameter, 12.8 mm vs. 8.8 mm, p=0.025; short diameter, 9.2 mm vs. 6.7 mm, p=0.041). The threshold of >10.0 mm for the long axis gave a sensitivity, specificity, positive predictive value, and negative predictive value of 87.5%, 70.6%, 58.3%, and 92.3%, respectively, using a binary classification test. Decision tree analysis revealed a sensitivity, specificity, and accuracy of 87.5%, 70.6%, and 76.0%, respectively, with the threshold of >10.0 mm for the long axis on MRI. The criteria of >10.0 mm for the long axis on MRI predicted an advanced stage and poorer prognosis using a validation set of 15 cases (p=0.028). CONCLUSION: Minimally invasive surgery after preoperative evaluation on MRI and SLN biopsy is a feasible strategy for patients with vulvar cancer.
Adult ; Aged ; Aged, 80 and over ; Female ; *Groin ; Humans ; Lymph Node Excision ; Lymphatic Metastasis/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Prognosis ; Retrospective Studies ; Sensitivity and Specificity ; Sentinel Lymph Node/*pathology ; Sentinel Lymph Node Biopsy/*methods ; Vulvar Neoplasms/*pathology/surgery

Carcinoma in Situ ; epidemiology ; etiology ; pathology ; Carcinoma, Squamous Cell ; pathology ; surgery ; Disease Progression ; Female ; Humans ; Lichen Sclerosus et Atrophicus ; epidemiology ; etiology ; pathology ; Prevalence ; Vulvar Neoplasms ; epidemiology ; etiology ; pathology

Adult ; Female ; Humans ; Middle Aged ; Myxoma ; pathology ; surgery ; Vulvar Neoplasms ; pathology ; surgery

OBJECTIVETo study the clinicopathologic features of primitive neuroectodermal tumor (PNET) in female genital tract.

METHODSSix cases of PNET arising in female genital tract were retrospectively reviewed. The clinicopathologic features, immunohistochemical findings and EWS gene translocation study results were analyzed.

RESULTSThe age of patients ranged from 10 to 27 years (mean = 20 years). The sites of involvement included ovary (1 case), uterus (1 case), vulva (2 cases) and vagina (2 cases). The greatest diameter of the tumor ranged from 2 to 10 cm (mean = 5.4 cm). The tumor had nodular appearance and showed grayish-pink fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Histologically, the tumor was composed of malignant small round cells with indistinct cell borders, hyperchromatic nuclei, dense chromatin, tiny nucleoli and scanty cytoplasm. The tumor cells were arranged in sheets or lobules. Homer-Wright rosettes were identified in 1 case. Immunohistochemical study showed that the tumor cells were positive for CD99, FLI-1 and CD56 (6/6). Focal expression of vimentin (5/6), NSE (5/6), nestin (4/6), synaptophysin (4/6), S-100 protein (2/6) and chromogranin A (1/6) was also demonstrated. EWS gene translocation was detected in 5 cases studied. Follow-up information was available in 2 patients (7 and 17 months of follow up, respectively). One of them died of tumor metastasis 17 months after diagnosis. The other patient was still alive.

CONCLUSIONSPNET arising in female genital tract is rare. It mainly involves ovary, uterus, vulva and vagina. Immunohistochemical study using a panel of antibodies and fluorescence in-situ hybridization play an important role in definitive diagnosis of this rare malignancy.

12E7 Antigen ; Adolescent ; Adult ; Antigens, CD ; metabolism ; CD56 Antigen ; metabolism ; Cell Adhesion Molecules ; metabolism ; Child ; Female ; Follow-Up Studies ; Genital Neoplasms, Female ; genetics ; metabolism ; pathology ; surgery ; Humans ; Neuroectodermal Tumors, Primitive, Peripheral ; genetics ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Proto-Oncogene Protein c-fli-1 ; metabolism ; RNA-Binding Protein EWS ; genetics ; Retrospective Studies ; Translocation, Genetic ; Uterine Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vaginal Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Vulvar Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Young Adult

Adult ; Aged ; Breast Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Female ; Gene Amplification ; Genital Neoplasms, Male ; genetics ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary ; genetics ; metabolism ; pathology ; surgery ; Paget's Disease, Mammary ; genetics ; metabolism ; pathology ; surgery ; Penile Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Receptor, ErbB-2 ; genetics ; metabolism ; Scrotum ; Vulvar Neoplasms ; genetics ; metabolism ; pathology ; surgery

Adult ; Female ; Humans ; Myxoma ; diagnosis ; pathology ; surgery ; Vulvar Neoplasms ; diagnosis ; 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

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 investigate the clinicopathological characteristics, diagnosis and differential diagnoses of proximal-type epithelioid sarcoma (PES).

METHODSFive cases of PES were retrieved from pathology files. Clinical, pathologic and immunohistochemical features of the tumors were reviewed.

RESULTSOne 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.

CONCLUSIONDefinitive diagnosis of PES relies on its histopathological characteristics in conjunction with appropriate immunohistochemical findings.

Adult ; Chemotherapy, Adjuvant ; Epithelioid Cells ; metabolism ; pathology ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Pelvic Neoplasms ; metabolism ; pathology ; surgery ; Radiotherapy, Adjuvant ; Sarcoma ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Vulvar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult ; beta Catenin ; metabolism