Adenoid cystic carcinoma of Bartholin's Gland. Case report with review of the literature

Adenoid cystic carcinoma of Bartholin's gland is characterized by slow growth, local invasion, and sometimes distant metastasis. There is no consensus regarding the optimal treatment. We report herein a rare case of stage II adenoid cystic carcinoma of Bartholin's gland that was successfully treated by wide local excision of the tumor and ipsilateral inguinal and pelvic lymphadenectomy. A 54-year-old Japanese woman was admitted complaining of painless, gradually increasing vulvar mass existing from ten years before. Local examination revealed a 3 x 2-cm hard mass in the left labium major. Pathological examination of subsequent excisional biopsy revealed adenoid cystic carcinoma originating in the Bartholin's gland. A wide local resection of the tumor with inguinal and pelvic lymphadenectomy was performed. The tumor was completely excised with free margins. There was no metastasis in the resected lymph nodes. The patient was diagnosed as having stage II vulvar cancer according to the International Federation of Gynecologists and Obstetricians classification (1988). The patient is now healthy without evidence of recurrence at 5 years after surgery. Adenoid cystic carcinoma of Bartholin's cyst at early stage can be treated by wide local excision as a primary surgery.     

Malignant eccrine spiradenoma of the vulva: a case report and review of the literature

Malignant eccrine spiradenoma is a rare skin tumor of sweat gland origin. We present the first reported case of this tumor in the female genitalia. Due to the rarity of this tumor, there has yet to be an established standard of care. The present case is that of a 41-year-old woman with malignant eccrine spiradenoma of the periclitoral region. She had an 18-month history of a recurrent, painful mass adjacent to the clitoris. Her diagnosis was made after excision of the cystic tumor. The patient then underwent a partial radical vulvectomy with bilateral sentinel lymph node sampling. As malignant eccrine spiradenoma is a rare tumor, no standard care exists for treatment and postoperative management. Based on our review of the literature, wide local excision appears to be the preferred initial treatment. Furthermore, adjuvant chemotherapy and/or radiation does not seem to improve survival in patients with advanced or recurrent cancer. Although lymph node sampling and/or lymphadenectomy is frequently reported in the treatment of this tumor, hematogenous metastasis can also occur. Therefore, these patients require close postoperative follow-up for recurrent disease.     

Sentinel node metastasis in the groin detected by technetium-labeled nannocolloid in a patient with cervical cancer

OBJECTIVE: The aim of this study was to describe the first sentinel groin node metastasis detected by technetium-labeled nanocolloid in a patient with cervical carcinoma. METHOD: Preoperatively, 60 mBq technetium-labeled nannocolloid was injected at 3 and 9 o'clock in the uterine cervix. Sentinel nodes were detected using a handheld and laparoscopic probe (Navigator) and removed for pathological assessment. RESULTS: A 52-year-old diagnosed with FIGO stage IIA squamous cervical carcinoma was referred to our unit. On physical examination a bulky cervical tumor and a 1.5-cm enlarged left inguinal lymph node were found. No other abnormalities were seen on pelvic MRI scan and CT scan of the abdomen and lower pelvis. Preoperative lymphoscintigraphy showed that a left groin node and three nodes located in the right obturator fossa were the sentinel nodes. They were easily detected using, respectively, a hand-held and a laparoscopic probe and removed. As both the inguinal and the obturator lymph nodes contained metastatic deposits, the patient was treated with the combination of chemotherapy and radiotherapy. CONCLUSION: Inguinal lymph nodes can rarely be the sentinel nodes in patients with cancer of the uterine cervix.     

Microcystic adnexal carcinoma of the vulva

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor. CASE: A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. Physical examination revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively. CONCLUSION: Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. Groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.     

Inguinal lymph node metastasis as the only manifestation of lymphatic spread in ovarian cancer: A case report

OBJECTIVE: Inguinal metastasis is a rare manifestation of ovarian cancer. Autopsy studies have reported inguinal metastasis in 0-3% of patients with advanced disease. CASE REPORT: We describe a 43-year-old patient with ovarian cancer limited to the adnexa who had an isolated metastasis in an enlarged inguinal lymph node. The patient underwent total abdominal hysterectomy, omentectomy, pelvic and paraaortic lymphadenectomy, and excision of the enlarged inguinal lymph node. All 78 pelvic and 40 paraaortic lymph nodes were negative. CONCLUSION: This case demonstrates that early isolated distant lymph node metastasis, although rare, can occur in patients with ovarian cancer and may be a presenting symptom.     

Adenoid cystic carcinoma of the Bartholin’s gland: a rare tumor unmarked by persistent vulvar pain in a postmenopausal women

INTRODUCTION: Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare malignancy characterized by slow growth, local invasion and perineural infiltration. CASE: A 64-year-old postmenopausal woman presented with persistent vulvar pain. Local examination revealed a 2 x 2 cm painful vulvar nodule. Nodule was excised and ACC originating in the Bartholin's gland with positive resection margin was determined in pathological examination. We performed hemivulvectomy and ipsilateral inguinal lymph node dissection. Pathology showed that the resection margins were free of tumor and there was no lymph node metastasis. DISCUSSION: In cases of persistent vulvar pain without swelling, complete vulvovaginal evaluation should be done to prevent delay in diagnosis of Bartholin's gland cancer. Treatment modality must be tailored to each patient, though there is no consensus on the treatment.     

Pathologic evaluation of inguinal sentinel lymph nodes in vulvar cancer patients: a comparison of immunohistochemical staining versus ultrastaging with hematoxylin and eosin staining

OBJECTIVES: To evaluate the value of immunohistochemical (IHC) staining of inguinal sentinel lymph nodes (SLN) found to be negative for metastatic disease by ultrastaging with hematoxylin and eosin (H&E) staining. METHODS: An IRB approved study identified 29 patients who had undergone an inguinal sentinel lymph node dissection for squamous cell carcinoma of the vulva. All sentinel lymph nodes found to be negative for metastatic disease based on ultrastaging with H&E staining were reevaluated with pancytokeratin antibody (AE1/AE3) immunohistochemical (IHC) staining to detect micrometastasis. RESULTS: Twenty-nine patients with squamous cell carcinoma of the vulva underwent an inguinal sentinel node dissection. Nineteen patients had inguinal dissections negative for metastatic disease, 2 patients had bilateral inguinal metastasis, and 8 patients had unilateral inguinal metastasis. A total of 42 groin dissections with SLN biopsies were performed; 12 groins were positive for metastatic disease and 30 were negative based on ultrastaging with eosin and hematoxylin staining. A total of 107 sentinel lymph nodes (2.5 SLN per groin) were obtained, of which 18 SLN contained metastatic disease identified by ultrastaging and staining with H&E. Two SLN contained micrometastasis less than 0.3mm in size and 16 SLN contained metastasis greater than 2mm in size. Eighty-nine SLN found to be negative for metastasis by ultrastaging with H&E staining were also negative for micrometastasis on evaluation with pancytokeratin antibody AE1/AE3 IHC staining. CONCLUSIONS: The addition of immunohistochemical staining to ultrastaging with H&E staining in the pathologic evaluation of inguinal sentinel lymph nodes does not increase the detection of micrometastasis in patients with primary squamous cell carcinoma of the vulva.     

A stage Ia vulvar carcinoma with an inguinal lymph node recurrence after local excision. A case report and literature review

A patient with a stage Ia vulvar squamous cell carcinoma (< 1 mm invasion) is reported in which an inguinal recurrence one and a half years after partial radical vulvectomy and superficial inguinal lymph node sampling was noted. After the initial biopsy showing a tumor invading 0.3 mm into the stroma, residual tumor could not be shown in the vulvectomy specimen nor in the superficial lymph nodes. A review of the literature indicates that this is only the second reported case of stage Ia vulvar carcinoma with lymph node metastases.     

Sentinel lymph node identification in a primary ductal carcinoma arising in the vulva

Primary or metastatic breast-like carcinoma of the vulva is a rare event. Because of the similarity with breast ductal carcinoma, we think that the same principles used for treatment of orthotopic breast cancer can be applied, as well as the use of sentinel lymph node technique, which is widely accepted in the management of early-stage breast cancer. We report a 49-old-year postmenopausal woman who was referred to our institution after small biopsy of a 3.5- x 3-cm right vulvar tumor. Histopathologically, infiltration of the vulvar dermis by a ductal carcinoma of mammary gland type was reported. At operation, the sentinel node technique revealed two sentinel nodes in the right inguinal area. Although these nodes proved negative for malignancy, the patient underwent wide local excision of tumor and complete ipsilateral inguinofemoral lymphadenectomy. The remaining excised nodes were negative. Surgical specimen proved estrogen- and progesterone-positive receptors, the reason for which the patient received tamoxifen adjuvant therapy. This report represents the first case in the world literature of primary breast carcinoma arising in the vulva in which sentinel lymph node identification has been possible. Because of the rarity of this condition, the pathologic similarity of this tumor along with currently accepted guidelines for the management of breast cancer supports the possibility of local excision and sentinel lymph node identification as a possible alternative to inguinofemoral lymphadenectomy.     

The relation of intraabdominal findings and retroperitoneal lymph node metastasis in the epithelial ovarian cancer patients

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Primary myoepithelial carcinoma of the vulva and review of the literature

Myoepithelial carcinoma of the vulva is extremely rare, with only five cases reported. Here, we describe a case of vulvar myoepithelial carcinoma along with a review of the literature. The patient, a 49-year-old woman, was referred for a tumor on the right labium majora. She underwent a wide local excision and bilateral inguinal lymph node dissection. Pathological examination revealed an unencapsulated, infiltrative pattern, with solid, nested and trabecular components and areas with myxoid or hyalinized stroma. The tumor consisted of oval to round epithelioid cells with moderate nuclear pleomorphism. By immunohistochemistry, the tumor cells were diffusely positive for cellular adhesion molecule (CAM) 5.2, epithelial membrane antigen (EMA), S-100 protein, and vimentin and focally positive for carcinoembryonic antigen (CEA) and p63, while negative for alpha- smooth muscle actin (SMA). The tumor was diagnosed as a myoepithelial carcinoma of the vulva, with metastases to the bilateral inguinal lymph nodes. Following completion of adjuvant radiotherapy, the patient remained alive without any evidence of recurrence at 56 months. A review of six cases of this tumor (including the present case), demonstrated variable morphology with some overlapping features. Therefore, immunohistochemistry using a panel of epithelial and myogenic markers is essential for definitive diagnosis. Two cases had inguinal lymph node metastases and received adjuvant radiotherapy or concurrent chemoradiotherapy, which resulted in good local control. One case had lung metastasis and was successfully treated by chemotherapy. Given the rarity of this disease and its uncertain prognosis, no clinical trials have been conducted regarding the necessity of adjuvant therapy. Myoepithelial carcinomas of the vulva are extremely rare making case series the most viable means of optimizing diagnosis and therapy.     

Carcinoma of the vulva. Clinicopathologic factors involved in inguinal and pelvic lymph node metastasis

Eighty-five women with vulvar squamous cell carcinoma were subjected to radical vulvectomy with bilateral inguinal and femoral node dissection or to radical vulvectomy with bilateral inguinofemoral and deep pelvic node dissection. The association between lymph node status (metastatic or not) and several parameters was analyzed: tumor location, size and clinical stage; tumor thickness, histologic grade and mitotic index; blood vessel, lymphatic and perineural infiltration; and lymphocytic and plasma cell infiltrates. There were no metastases to the pelvic lymph nodes without previous inguinal lymph node involvement. Unilateral vulvar carcinomas did not have contralateral metastatic nodes when there was no ipsilateral nodal involvement. Lymphatic vessel infiltration showed a statistically significant correlation with inguinal node metastases (P less than .05). No correlation was found between lymph node metastasis and tumor size, clitoral invasion, tumor thickness, histologic grade, blood vessel and perineural infiltration, lymphocytic and plasma cell infiltrates, and mitotic index.     

Urachal adenocarcinoma metastatic to the ovaries: case report and literature review.

We report a urachal adenocarcinoma metastatic to both ovaries in a 50-year-old Japanese woman. Pelvic examination and imaging studies revealed a large cystic tumor occupying the pelvis and another cystic tumor between the umbilicus and the urinary bladder. A laparotomy was performed. Histopathological examination revealed a urachal tumor that was a well-differentiated invasive mucinous adenocarcinoma; the overlying urothelium was intact. The right and left ovarian tumors were well-differentiated mucinous adenocarcinomas. The urachal and ovarian tumors were immunoreactive for cytokeratin 20 and carcinoembryonic antigen, but negative for cytokeratin 7. The patient is alive with lymph node and bone metastases 6 months postoperatively. This is the eighth reported case of an adenocarcinoma of the bladder with ovarian metastasis.     

Risk factors for the development of lymph node metastasis in vulvar squamous cell carcinoma.

One hundred and ten women who underwent vulvectomy and inguinal-femoral lymphadenectomy for stages I-IV vulvar squamous cell carcinoma were studied. The most important factors that affected the inguinal lymph node status in the order of importance were vascular invasion, clinical stage, tumor thickness, depth of stromal invasion, and amount of keratin. Fourteen (88%) of 16 tumors with vascular invasion in the primary tumor metastasized. In the absence of vascular invasion, 18 (19%) of 94 tumors metastasized. Overall, 82% of tumors were correctly classified into lymph node negative and positive groups on the basis of vascular invasion. Tumor thickness and depth of stromal invasion had a similar accuracy in predicting lymph node status. The risk of lymph node metastasis increased from 0% when tumor thickness or depth of stromal invasion was less than 2 mm, to over 20% when depth of stromal invasion was greater than 2 mm, and to over 40% when tumor thickness exceeded 4 mm. A combination of vascular invasion, tumor thickness (or depth of stromal invasion), and the amount of keratin correctly classified 97% (76/78) of the lymph node negative group and 63% (20/32) of the positive group with an overall accuracy of 87%. The probability of having lymph node metastasis was computed for individual patients on the basis of one or more pathologic parameters using a logistic regression model. This feasibility is an important step toward individualized therapy for vulvar carcinoma.     

Rare type of metastases in progressive cervix carcinoma

There are demonstrated two cases of advanced cervical carcinoma with unusual localization of metastases. One patient showed a scalp metastasis and the other one had a ulcerated inguinal lymph node metastasis. X-ray therapy caused a sufficient remission of the metastatic tumors.     

Verrucous carcinoma of the vulva in Turner syndrome

BACKGROUND: Verrucous carcinoma of the vulva in a patient with Turner syndrome is extremely rare. CASE REPORT: A 7.5 x 4 cm exophytic tumor of the vulva with a palpable ipsilateral groin lymph node developed in a 41-year-old single nulligravid Japanese woman with short stature and a history of primary amenorrhea. Analysis of karyotype demonstrated mosaic Turner syndrome. She underwent total deep vulvectomy with en-block inguinal femoral lymphadenectomy. Histopathological finding of the extirpated specimen demonstrated verrucous carcinoma with clear margins and the absence of metastasis to the lymph nodes. There was no sign of HPV genome detected in the carcinoma tissue. CONCLUSION: This seems to be the first case of vulvar verrucous carcinoma in a patient with Turner syndrome.     

Small cell carcinoma of the ovary with hypercalcemia causes severe pancreatitis and altered mental status

BACKGROUND: Small cell carcinoma of the ovary is a rare, highly malignant tumor that often exhibits a paraneoplastic hypercalcemia. CASE: A 27-year-old female presented with pancreatitis and altered mental status with hypercalcemia. Further investigation revealed a left ovarian mass and a small cell carcinoma of the ovary, hypercalcemic type was found. Hysterectomy with bilateral salpingo-oophorectomy was performed, and the patient underwent chemotherapy with carboplatin and paclitaxel. Hypercalcemia resolved after tumor resection. The patient has retroperitoneal lymph node recurrence at 16 months. CONCLUSION: Paraneoplastic hypercalcemia may result in pancreatitis and delirium. Small cell carcinoma of the ovary should be considered under these circumstances.     

Malignant clear cell hidradenoma of the vulva: report of a unique case and review of the literature.

Clear cell hidradenoma (CCH) is a rare tumor derived from eccrine sweat glands with a predilection for the head, face, and upper extremities. Its biologic behavior is unpredictable, although frank malignant transformation is reportedly rare (prevalence rate, 6.7% in a review). Malignant CCH (MCCH) exists only as case reports or very small series in the literature. We present a unique case of MCCH of the vulva with completely bland cytological features. A previously healthy 39-year-old woman underwent marsupialization of a presumed left-side vulval Bartholin gland cyst. Microscopy revealed a tumor with the features of CCH; no atypia, necrosis, or mitoses were observed. Ten months later, she developed enlarging left groin nodes, one of which contained a metastatic clear cell tumor. Radiological examination did not reveal any other primary source, and the diagnosis of MCCH was confirmed through an expert review. A reexcision of the vulval primary site, which contained residual tumor, was performed. The patient is currently free of any further recurrence 10 months after the excision of the lymph node metastasis. The case highlights the difficulty in predicting the behavior of CCH on the basis of histological examination alone. It also highlights the importance of considering MCCH in the differential diagnosis of a lymph node containing a metastatic clear cell tumor or an apparent metastatic clear cell lesion in the skin. We review the current literature on MCCH and discuss the problems in the differential diagnosis and treatment of this rare tumor.     

A clinicopathological study of 30 melanomas of the vulva.

Thirty cases of malignant melanomas of the vulva were studied for prognostic factors. Ulceration, tumor thickness, and positive inguinal lymph nodes were the most important prognostic factors. Morphometry did not demonstrate any prognostic meaning. Traditionally a radical vulvectomy and bilateral inguinal lymph node dissection were the therapy of choice, but this treatment modality did not show a better survival than less radical treatment. A low-risk and a high-risk group of patients have been identified for recurrence. The low-risk patient has a nonulcerative tumor, less than 3 mm thick, without clinical evidence of inguinal lymph node metastases, and should be treated by local excision with a 2- to 3-cm margin. The high-risk patient has a tumor which is ulcerative and/or more than 3 mm thick and should also be treated by local excision without elective inguinal node dissection. If clinical suspicion of inguinal lymph node metastases exists, an inguinal node dissection is advocated for better local control of the disease.     

化疗对晚期卵巢、输卵管及腹膜浆液性癌淋巴结转移的临床疗效(附70例临床分析)

目的:探讨化疗对晚期卵巢、输卵管及腹膜浆液性癌腹膜后淋巴结转移的临床疗效。方法:将晚期卵巢、输卵管及腹膜浆液性癌70例患者,根据淋巴结切除术与化疗的先后顺序分为2组:A组21例,化疗后行淋巴结切除术。B组49例,淋巴结切除术后化疗。比较2组患者的一般情况、治疗方案及淋巴结转移情况。结果:2组患者的年龄,肿瘤分期分级,治疗前的肿瘤标记物,切除淋巴结的数量等均无显著性差异(P>0.05)。初治时A组患者盆腹腔黏连及转移重于B组;初次手术A组进行满意的肿瘤细胞减灭术的几率明显低于B组(0%vs73.47%,P<0.05)。化疗后A组患者淋巴结转移率低于B组(38.10%vs49.00%,P>0.05),腹主动脉旁淋巴结为肿瘤最常侵犯的淋巴结。A组行TC(泰素/紫杉醇+伯尔定/卡铂)或TP(泰素/紫杉醇+顺铂)方案化疗的患者淋巴转移率低于行CAP(顺铂+阿霉素+环磷酰胺)方案的患者(33.33%vs50%,P=0.045)。结论:化疗对卵巢、输卵管及腹膜浆液性癌淋巴结转移有一定的疗效。初治时不能进行淋巴结切除的患者可考虑先行化疗后再次手术切除淋巴结。