Case of lung carcinoma revealed by vulvar metastasis associated with systemic scleroderma and literature review

Metastatic carcinoma to the vulva is rare, where the incidence is believed to be between 5% and 8%. However, malignant tumors have been described in 3-11% of systemic scleroderma (SSc) cases. We report the case of one patient, a 66-year-old postmenopausal woman, whose medical history was marked with rheumatic vascular disease (systemic scleroderma) since 1993 without muscular, renal, cardiac lesions or HTA (arterial hypertension) and without tobacco history. The woman presented with a new vulvar mass of the right labia in December 2011 that had progressively enlarged in size. CT scan of the abdominopelvic region demonstrated a lobular mass of the right labia with central necrosis, 7 cm on the wide axis, and the rectum and the vaginal wall were normal. No inguinal or iliac lymphadenopathy was noted. An outpatient excisional biopsy revealed a poorly differentiated malignant tumor suggestive of carcinoma. IHC: CK7+/CK20-, estrogen receptors-, AE 1 AE 3+, vimentine+, S100-, Desmina-, CD34-, KI 67: 20%. The thoracic scan revealed a large mass of 4 cm × 3 cm in the right lung base with right paratracheal lymphadenopathy 3 cm × 2 cm. A bronchoscopy revealed discrete stenosis of the mediastinal portion of the right bronchial tree. The bronchial biopsy also revealed poorly differentiated lung carcinoma, non-small cell, which was identical with the vulvar tumor.

The presence of the single lung lesion with only one lymphadenopathy paratracheal with pathological and immunohistochemical (IHC) profile similar to the vulvar lesion, and a particular IHC profile with CK7+ and CK20- was detected - that is more specific to the primitive pulmonary cancer, and the presence of only one sarcoma marker vementine+, desmine and actine-. Also the presence of KI 67: 20%, predicted the proliferative and great metastatic power of the lung tumor was observed. Additionally, lung cancer was the most frequent type and may develop in scleroderma as reported in most studies. This allows to conclude for primitive lung carcinoma revealed with vulvar metastasis after elimination of the possibility of vulvar sarcoma. The patient was treated by chemotherapy (Taxol/Platin) with partial response from the lung after 3 cycles and palliative radiotherapy in the vulva with a good response. This case described primary lung carcinoma associated with scleroderma, revealed by a vulvar metastasis, which may be related to the aggressiveness of lung cancer when the lung fibrosis follow-up is not performed well to detect early the development of lung tumors in the patient with systemic scleroderma.