Deguidi Giulia, Mirandola Sara, Nottegar Alessia, Tsvetkova Vassilena, Bianchi Beatrice, Pellini Francesca
Complex Operative Unit of Breast Surgery - Breast Unit AOUI, Verona, Italy.
Department of Pathology and Diagnostics- Breast Unit AOUI, Verona, Italy.
Int J Surg Case Rep. 2020;77:834-838. doi: 10.1016/j.ijscr.2020.11.073. Epub 2020 Nov 27.
The present study reports the case of an axillary hibernoma in a patient with lobular homolateral breast cancer and multiple endocrine neoplasia type1 (MEN-1). Hibernoma is a rare benign adipose tissue tumor, and usually manifests as a slowly growing and painless rubbery mass. These tumors can arise in various sites, but mammary hibernomas remain extraordinarily uncommon. Although hibernomas are metabolically active and therefore "glucose-avid" on fluorodeoxyglucose CT-positron emission tomography (FDG CT-PET), imaging alone is inadequate in providing a reliable diagnosis and definitive differential diagnosis from other malignancy. Only complete surgical excision is diagnostic and, in most cases, curative.
A 42-years-old woman was followed for MEN-1 syndrome associating with hyperparathyroidism, insulinoma, non-secretory adrenal adenoma and thyroid lump. A FDG CT-PET found high glucid hypermetabolism in thickened elongated area on the front axillary line. Hibernoma was diagnosed after realization of prophylactic left mastectomy, homolateral sentinel lymph node biopsy and exeresis of the known axillary lesion.
Clinical importance lies in distinguishing hibernoma from other benign and malignant breast neoplasms, as well as inflammatory conditions that come into the histologic or radiologic differential. Hibernoma is not currently classified as a non-endocrine tumor related to MEN1, but this association could be not fortuitous for the linkage between modification of Menin protein function and pathogenesis of hibernomas.
Our case deserves extraordinary attention because, not only it's a case of MEN1 syndrome associated with hibernoma, but in the context of this lesion there are multiple micro-foci of infiltrating lobular carcinoma.
本研究报告了一例患有小叶型同侧乳腺癌和多发性内分泌腺瘤1型（MEN-1）患者的腋窝冬眠瘤病例。冬眠瘤是一种罕见的良性脂肪组织肿瘤，通常表现为生长缓慢且无痛的橡皮样肿块。这些肿瘤可发生于各种部位，但乳腺冬眠瘤仍然极为罕见。尽管冬眠瘤代谢活跃，因此在氟脱氧葡萄糖CT-正电子发射断层扫描（FDG CT-PET）上表现为“葡萄糖摄取增加”，但仅靠影像学检查不足以提供可靠的诊断以及与其他恶性肿瘤的明确鉴别诊断。只有完整的手术切除才具有诊断意义，并且在大多数情况下可治愈。
一名42岁女性因MEN-1综合征接受随访，该综合征伴有甲状旁腺功能亢进、胰岛素瘤、无功能性肾上腺腺瘤和甲状腺肿块。FDG CT-PET检查发现腋前线增厚延长区域有高糖代谢。在进行预防性左乳房切除术、同侧前哨淋巴结活检以及切除已知的腋窝病变后，诊断为冬眠瘤。
临床重要性在于将冬眠瘤与其他良性和恶性乳腺肿瘤以及组织学或放射学鉴别诊断中涉及的炎症性疾病区分开来。目前冬眠瘤未被归类为与MEN1相关的非内分泌肿瘤，但这种关联可能并非偶然，因为Menin蛋白功能改变与冬眠瘤发病机制之间存在联系。
我们的病例值得特别关注，因为它不仅是一例与冬眠瘤相关的MEN1综合征病例，而且在该病变背景下存在多个浸润性小叶癌微灶。
