Pusiol T, Morichetti D, Zorzi M G
Institute of Anatomic Pathology, S. Maria del Carmine Hospital Rovereto (TN), Italy.
Pathologica. 2011 Jun;103(3):64-7.
Sebaceous glands are abundant on the vulva, but vulvar sebaceous carcinoma (SC) is an uncommon neoplasm.
We report a case of SC of the vulva in a 51 year-old woman.
The patient presented a 6-month history of an asymptomatic 2.5 x 1.5 cm exophytic tumour localized on the left labium majora. Tumorectomy was performed. Histologically, the lesion had an irregular lobular growth pattern composed of lobules or sheets of malignant cells separated by fibrovascular stroma. There was a mixture of sebaceous-type differentiation, small ducts and areas showing basaloid or squamous features. Centrally-located tumour cells showed moderate EMA immunoreactivity, especially enhancing cytoplasmic "bubbliness". Tumour cells were immunoreactive for CAM 5.2. The immunoreactivity for intranuclear p53 staining was > 10%. Southern blot hybridization and PCR studies did not detect HPV DNA. Hemivulvectomy was performed. After 18 months of follow-up, the patient has no evidence of recurrence, metastases or other malignant tumours.
The grading of cutaneous SC proposed by Rutten et al. (World Health Organization Classification of Skin Tumours) and Patterson & Wick (Nonmelanocytic Tumours of the Skin. Armed Forces Institute of Pathology) is based on patterns of tumour growth rather than cytological features. Such grading of skin SC, including vulvar SC, should not be used since its prognostic value has not been sufficiently documented. As the number of reported vulvar SCs is very limited, their natural history is unknown and the optimal treatment has not been established. The follow-up of 7 reported cases supports the general opinion that the tumour may be aggressive. SC groin node metastases carry a devastating prognosis, and unrecognized disease in the inguinofemoral lymph nodes is nearly always fatal. The use of sentinel lymph nodes (SLN) has evolved as an effective surgical technique for identifying early subclinical regional nodal involvement for many solid tumours throughout the body for staging disease; this is because extra-ocular SCs cause widespread metastatic disease. In our opinion, SLN should be used in conjunction with wide local excision of the primary tumour to investigate regional subclinical metastases. In the presence of a positive sentinel node, early lymphadenectomy with or without radiotherapy could be used to reduce tumour-related morbidity and mortality. The histopathologic differential diagnosis of SC is wide-ranging, including virtually all other malignant clear cell tumours of the skin. The proliferative pattern, immunostaining and cytologic features permit exclusion of neoplasms that mimic SC, but a diagnosis of SC should be rendered only if the overall attributes of the lesion are appropriate for such a interpretation.
外阴皮脂腺丰富，但外阴皮脂腺癌（SC）是一种罕见的肿瘤。
我们报告一例51岁女性的外阴皮脂腺癌病例。
患者有一个位于左侧大阴唇的无症状外生性肿瘤，病史6个月，大小为2.5×1.5 cm。进行了肿瘤切除术。组织学上，病变呈不规则小叶状生长模式，由被纤维血管间质分隔的小叶或恶性细胞片组成。存在皮脂腺型分化、小导管以及显示基底样或鳞状特征的区域的混合。位于中央的肿瘤细胞显示中度EMA免疫反应性，特别是增强了细胞质的“泡状”。肿瘤细胞对CAM 5.2呈免疫反应性。核内p53染色的免疫反应性>10%。Southern印迹杂交和PCR研究未检测到HPV DNA。进行了半侧外阴切除术。随访18个月后，患者无复发、转移或其他恶性肿瘤的证据。
Rutten等人（《世界卫生组织皮肤肿瘤分类》）和Patterson与Wick（《皮肤非黑素细胞肿瘤。武装部队病理研究所》）提出的皮肤皮脂腺癌分级基于肿瘤生长模式而非细胞学特征。不应使用这种皮肤皮脂腺癌的分级，包括外阴皮脂腺癌，因为其预后价值尚未得到充分证明。由于报道的外阴皮脂腺癌病例数量非常有限，它们的自然病史尚不清楚，最佳治疗方法也未确定。对7例报道病例的随访支持了肿瘤可能具有侵袭性的普遍观点。皮脂腺癌腹股沟淋巴结转移预后不佳，腹股沟股淋巴结未被识别的疾病几乎总是致命的。前哨淋巴结（SLN）的应用已发展成为一种有效的手术技术，用于识别全身许多实体肿瘤的早期亚临床区域淋巴结受累情况以进行疾病分期；这是因为眼外皮脂腺癌会导致广泛的转移性疾病。我们认为，SLN应与原发肿瘤的广泛局部切除联合使用，以研究区域亚临床转移情况。在前哨淋巴结阳性的情况下，可使用早期淋巴结清扫术联合或不联合放疗来降低肿瘤相关的发病率和死亡率。皮脂腺癌的组织病理学鉴别诊断范围广泛，几乎包括皮肤的所有其他恶性透明细胞肿瘤。增殖模式、免疫染色和细胞学特征有助于排除模仿皮脂腺癌的肿瘤，但只有当病变的整体特征适合这种解释时，才能做出皮脂腺癌的诊断。
