Axillary cavernous lymphangioma in pregnancy and puerperium

BACKGROUND: Cavernous lymphangioma is a form of benign congenital abnormality grouped under lymphatic malformations, which can clinically manifest as a cystic tumor. Common areas are the neck and axillary region. Most cases are diagnosed in children under the age of 2, and occasionally the diagnosis is made in utero. CASE: We present the case of a 29-year-old primigravid woman with an axillary cavernous lymphangioma which rapidly increased in size during pregnancy, and describe the treatment plan in this unusual situation. CONCLUSION: The treatment of a large lymphangioma during pregnancy has to be well planned, considering the welfare of the mother and baby. Most of the current therapies have never been tested in pregnant or lactating women. We discuss the possible pathomechanisms for the growth of lymphangioma during pregnancy with the overproduction of cytokines such as vascular endothelial growth factor.     

Malignant fibrous histiocytoma of the vulva: a case report

Background: Primary sarcomas of the vulva are rare tumors that account for 1.8–3% of all vulvar malignancies. Malignant fibrous histiocytoma occurs infrequently on the vulva but nonetheless is the second most frequent sarcoma of this region. The purpose of this report is to review the diagnosis and therapy of this exceedingly rare tumor. Case: A 72-year-old woman was presented with a vulvar mass that was ultimately found to be a vulvar malignant fibrous histiocytoma. After surgical excision and 9 months of initial diagnosis, she returned with rapid localized progression and pulmonary metastases. Conclusion: Malignant fibrous histiocytoma arising in the vulva represents an example of vulvar sarcomas. Since the diagnostic criteria and natural history of this tumor still remain poorly defined, further case studies may be helpful to elucidate these issues as well as the optimal therapeutic approach of this heterogeneous group of malignancies.     

Extraskeletal myxoid chondrosarcoma: a report of a gynecologic case

BACKGROUND: Extraskeletal myxoid chondrosarcoma is a rare tumor that has been reported and discussed in neurosurgical, orthopedic, and otolaryngologic literature. We report a case of vulvar extraskeletal myxoid chondrosarcoma. CASE: This is a case of a 46-year-old woman who presented with a soft tissue mass in the left vulva and underwent local excision. Grossly, the tumors appeared to be leiomyomas. Pathology revealed an extraskeletal myxoid chondrosarcoma. CONCLUSION: We report the first case of an extraskeletal myxoid chondrosarcoma arising in the vulva.     

Fibroma of the vulva: a case report

BACKGROUND: It is rare to see large vulvar growths that are not trauma related in young women. The largest vulvar mass recorded dates back to 1851. Fibroma of the vulva is not common but, if not seen early and removed, can be emotionally draining for the patient. CASE: A large vulvar mass was found in an 18-year-old woman and was expeditiously excised. The patient was emotionally withdrawn and would not participate in any school or social activities. She would carry moist paper towels with her to prevent maceration of the lower abdomen and lesion wall. CONCLUSION: An extraordinary physical finding can embarrass and alter a patient's demeanor and should be addressed without delay. The emotional condition of our patient prior to surgery consisted of depression and emotional stress due to the large vulvar mass. Following surgery the patient was comfortable, and her demeanor is now excellent.     

Primary yolk sac tumor (endodermal sinus tumor) of the vulva: case report and review of the literature

INTRODUCTION: Endodermal sinus tumor (EST) or primary yolk sac tumor (YST) of the vulva is extremely rare and a highly malignant germ cell tumor. Only nine cases of vulvar YST have been reported to the world literature to date. We present the tenth case of endodermal sinus tumor of the vulva. CASE: A 32-year-old white virgin presented with a 3.5 cm right labial mass without any other signs or symptoms. Excisional biopsy showed YST with a predominantly solid pattern. Unilateral hemivulvectomy with bilateral inguinal lymphadenectomy was performed. Six months after surgery there was a recurrence. She was treated with three courses of the BEP regimen (bleomycin, etoposide, cisplatin). The patient refused to take any further treatment including radiotherapy. The serum alpha-fetoprotein (AFP) was not elevated at the initial diagnosis however it was elevated during recurrence. The patient is alive with the disease 42 months after the first appearance of the vulvar mass.     

Vulvar Hibernoma: An Unusual Lipomatous Tumor in an Adolescent

BackgroundLipomatous tumors of the vulva are exceedingly rare, particularly in adolescents. We describe the work-up and management of an adolescent girl who presented with a large, well-vascularized vulvar mass.CaseA 14-year-old girl presented with a large vulvar mass of unclear etiology. Magnetic resonance imaging of this mass revealed an ill-defined, well-vascularized mass with fat signal characteristics suggestive of a lipomatous tumor that was concerning for malignancy. We performed complete resection of the mass, and histologic evaluation revealed a vulvar hibernoma. There have been no signs of recurrence 1 year later.Summary and ConclusionAlthough rare, a hibernoma of the vulvar region may present in adolescence and may be concerning for malignancy on imaging. Complete resection of these tumors is recommended for definitive diagnosis and treatment.     

Myeloid sarcoma of the vulva as the presenting symptom in a patient with acute myeloid leukemia

BACKGROUND: Myeloid sarcoma is a tumor of myeloblasts or immature myeloid cells occurring in an extramedullary site. Myeloid sarcoma of female genital tract is very rare with no cases of vulvar location reported in English-language literature. CASE: A 73-year-old female presented with an indurated mass encompassing her left labia majora and vulva. The mass was diagnosed as vulvar myeloid sarcoma. The patient's peripheral blood smear revealed Auer rods and other findings consistent with a diagnosis of acute myeloid leukemia (M-2 type, FAB classification). CONCLUSION: To the best of our knowledge this case is the first report in the English-language literature of the myeloid sarcoma of the vulva. Correct diagnosis of myeloid sarcoma in an otherwise asymptomatic patient is crucial for early administration of antileukemic chemotherapy.     

Primary lung large cell carcinoma metastatic to the vulva: a case report and review of the literature

BACKGROUND: Metastatic cancer to the vulva is a rare diagnosis and is estimated to account for 5-8% of all vulvar cancers. CASE: A 57-year-old postmenopausal woman was referred for the evaluation of a new vulvar mass. CT scan of the pelvis demonstrated a 4 x 6 cm lobular mass of the left labia. An outpatient excisional biopsy revealed a poorly differentiated large cell carcinoma with prominent necrosis and focal perineural invasion consistent with a bronchogenic carcinoma. A subsequent PET scan of the chest revealed a large primary lung carcinoma confirmed by CT-guided biopsy that was identical to the vulvar tumor. CONCLUSION: This case represents the only literature described primary lung carcinoma presenting as a vulvar metastasis.     

Squamous cell carcinoma with sarcomatoid features of the vulva: a case report and review of literature

BACKGROUND: A squamous cell carcinoma with sarcomatoid features of the vulva is an extremely rare malignancy of the female genital tract. This type of tumor is known to grow rapidly and associated with poorer clinical outcomes than those of squamous cell carcinoma of the vulva. CASES: A 43-year-old woman presented to our institute with a 4-month history of an aggravated vulvar mass. A radical local excision, bilateral inguinal lymph node dissection and laparoscopic assisted vaginal hysterectomy were performed. The FIGO stage of the vulvar cancer was stage II (T(2)N(0)M(0)) and the pathologic finding was consistent with a poorly differentiated squamous cell carcinoma with extensive sarcomatoid features. No further treatment was given and there was no clinical evidence of recurrence during the 2 years of follow-up. CONCLUSION: A squamous cell carcinoma with sarcomatoid features of the vulva is a tumor with aggressive biological behavior. To date, there have been only 15 cases of this disease reported in the literature. So, a collection and close study of these cases would be extremely useful in singling out and identifying the best treatment possible for this type of tumor.     

Supernumerary Nipple Presenting as a Vulvar Mass in an Adolescent: Case Report and Literature Review

BackgroundEctopic breast tissues can be found along the embryonic mammary ridges and can occur in the vulva. While ectopic breast tissue is not uncommon, functional breast with overlying nipple located within the vulva is exceedingly rare.CaseA 17-year-old with undiagnosed hypothyroidism presents with vulvar mass draining milky white fluid. A small lesion with appearance similar to a skin tag is noted and milky fluid expressed. Biopsy and excision of this mass confirmed the presence of a functional supernumerary nipple.Summary and ConclusionsThis appears to be the first reported case of a supernumerary nipple with symptomatic lactation in a non-pregnant adolescent. Supernumerary nipple should be considered in the differential diagnosis of a vulvar mass. Ectopic breast tissue in the vulva can undergo malignant transformation, therefore excision of this tissue is generally recommended.     

Sarcoma in association with multimodality management of vulvar cancer: two case reports

BACKGROUND: Radiation-induced or -associated sarcoma is a rare event which has been well described in the literature. However, this entity has been infrequently described in association with genital tract malignancies. To our knowledge it has never been described in association with the management of vulvar cancer. CASES: . Two different cases of sarcoma developing after primary management for vulvar cancer are presented, the first being a case of angiosarcoma developing in the lower abdominal wall 36 months after initial therapy and the second a case of fibrosarcoma developing on the vulva 7 years after multimodality treatment. CONCLUSION: Radiation-associated sarcoma after treatment for vulvar carcinoma is a rare event. A multimodality treatment of carcinoma of the vulva should not be withheld because of fear of sarcomagenesis.     

Leiomyosarcoma of the vulva: a case report

BACKGROUND: Leiomyosarcoma of the vulva is a rare gynecologic malignancy, comprising approximately 1% of vulvar cancers. CASE: A 36-year-old woman was referred for a slowly growing painless vulvar mass that was initially thought to be a Bartholin's duct cyst but was cancerous upon biopsy. A modified radical vulvectomy was performed, and pathology revealed a grade 1 leiomyosarcoma. Thirteen months later, the tumor had not recurred. CONCLUSION: Vulvar cancer must be considered in patients with a suspected Bartholin duct cyst that demonstrates atypical features. A biopsy should be obtained if the mass appears firm or solid on palpation, is ulcerated or presents in a slightly different location from the usual area of the Bartholin gland.     

Yolk sac tumor of the vulva: a case report with long-term disease-free survival

BACKGROUND: Yolk sac tumor (YST) of the vulva is extremely rare. Seven cases of vulvar YST have been reported to the literature. Due to the rarity of tumors, the appropriate choice of treatment may remain unclear. CASE: A 30-year-old woman presented with a 3.5-cm right labial mass. Excisional biopsy showed YST with predominant solid pattern. Three weeks after excision, right inguinal lymph node biopsy revealed metastatic tumor. The serum alpha-fetoprotein (AFP) was not elevated. Cisplatin-based chemotherapy was administered, followed by pelvic and groin irradiation. The patient was free of disease 90 months after the diagnosis. CONCLUSION: Local excision of tumor with adjuvant cisplatin-based chemotherapy can be justified for vulvar YST. Inguinal lymphadenectomy is recommended because metastasis may occur early. Adjuvant radiation therapy may help to control the disease. Tumor size of 5 cm or less may be a favorable prognostic factor. Serum AFP level may not be a sensitive marker for follow-up of vulvar YST.     

Myxoid leiomyosarcoma of the vulva

BACKGROUND: Myxoid leiomyosarcoma (MLMS) of the vulva is a mesenchymal tumor with only five reported cases in the literature. CASE: We report an 85-year-old woman with a unilateral nonulcerating, painless vulvar mass. According to the patient, the mass slowly enlarged. Initial biopsies were benign. However, clinically, this lesion was suspicious for a soft tissue tumor. Therefore, the mass was removed by a wide local excision. Definitive histology revealed a myxoid leiomyosarcoma of the vulva. At present, 25 months after the operation, the patient is well with no sign of recurrence. CONCLUSIONS: Vulvar myxoid leiomyosarcomas are rare and can be confused with other benign or malignant tumors. It is important to be aware of this rare tumor variant, in order to plan appropriate treatment.     

Pregnancy-associated invasive squamous cell carcinoma of the vulva in a 28-year-old, HIV-negative woman. A case report

BACKGROUND: The incidence of invasive squamous cell carcinoma of the vulva in women under 40 years of age has been increasing, particularly in association with human papillomavirus. Invasive vulvar carcinoma is rare in women under 30, as is an association with pregnancy. We report on a 28-year-old woman who was diagnosed with invasive squamous cell carcinoma of the vulva during pregnancy. CASE: The patient, gravida 5, para 4105, HIV negative, presented to the emergency room with vulvar pain. She had delivered a term infant three months earlier at another institution and was diagnosed with squamous cell carcinoma of the vulva at that time. At this admission, a 4.0-cm, ulcerated lesion involving the left labium minus was noted. The patient underwent examination under anesthesia with bilateral inguinal lymph node dissection, cone biopsy, radical vulvectomy and excision of perianal lesions. CONCLUSION: This case demonstrates the need to biopsy all suspicious vulvar lesions, even in young and pregnant women.