Sanchez Saz Jaime, Sanz Medrano Santiago, Nielsen Luna, Buendia Perez Javier, Manrique Gamo Elena, Cebrian Parra Juan Luis
Department of Orthopaedic Oncology, Orthopaedic Surgery and Traumatology, Hospital Clinico San Carlos, Madrid, Spain.
Department of Plastic and Reconstructive Surgery, Hospital Clinico San Carlos, Madrid, Spain.
J Orthop Case Rep. 2022;12(5):49-53. doi: 10.13107/jocr.2022.v12.i05.2812.
Hidradenocarcinomas are very rare and aggressive soft-tissue tumors, originated from sweat gland cells, which are located most frequently in head and neck, being their appearance at the extremities rare. This kind of tumor usually appears de novo and very few cases have been reported until now of appearance over benign lesions such as hidradenomas. Malignancy progression rate of hidradenomas is unknown, and this benign lesion has clinical and histopathological characteristics in common with hidradenocarcinomas that could lead to misdiagnosis. Hidradenocarcinomas have a very poor survival rate; therefore, an early diagnosis is essential for a better prognosis, and that is the reason why hidradenomas should be widely excised from the beginning, instead of performing marginal resections of this lesions that could lead to an aggressive recurrence.
Here is a case report of a 27-year-old woman diagnosed with a hidradenocarcinoma over a previously excised hidradenoma in the right foot. The diagnosis was made after right pelvic and inguinal lymphadenopathies appeared few months after a new small asymptomatic lump appeared at the same place in the sole of the right foot were the excised hidradenoma five years before was located. Lymph node biopsy was performed, with histopathological diagnosis of hidradenocarcinoma metastasis. Surgical local wide excision of the lump at the foot and lymphadenectomies was performed. Histopathological analysis of the samples confirmed the diagnosis of hidradenocarcinoma. The patient later received adjuvant radiotherapy and after one year there are no signs of disease recurrence.
Many questions remain uncertain about the management and treatment of hidradenocarcinomas due to the rarity of this type of tumor. Although targeted molecular therapies have shown promising results, more studies in this field are necessary. An early diagnosis and differentiation from its benign counterparts that allow local control of the disease before spreading is essential to improve survival rates.
汗腺癌是非常罕见且具有侵袭性的软组织肿瘤,起源于汗腺细胞,最常发生于头颈部,极少出现在四肢。这种肿瘤通常为原发性,迄今为止,仅有少数病例报道其出现在诸如汗腺腺瘤等良性病变之上。汗腺腺瘤的恶性进展率尚不清楚,且这种良性病变在临床和组织病理学特征上与汗腺癌有共同之处,可能导致误诊。汗腺癌的生存率极低;因此,早期诊断对于改善预后至关重要,这就是为什么从一开始就应广泛切除汗腺腺瘤,而不是对这种病变进行边缘切除,否则可能导致侵袭性复发。
本文报告一例27岁女性病例,其在先前切除的右脚汗腺腺瘤上被诊断为汗腺癌。诊断是在右脚足底原切除汗腺腺瘤的部位出现一个新的无症状小结节数月后,右侧盆腔和腹股沟淋巴结肿大时做出的。进行了淋巴结活检,组织病理学诊断为汗腺癌转移。对足部肿块进行了手术局部广泛切除及淋巴结清扫。样本的组织病理学分析证实了汗腺癌的诊断。患者随后接受了辅助放疗,一年后无疾病复发迹象。
由于这种肿瘤罕见,关于汗腺癌的管理和治疗仍有许多问题尚不确定。尽管靶向分子疗法已显示出有前景的结果,但该领域仍需要更多研究。早期诊断并与良性对应物区分开来,以便在疾病扩散前进行局部控制,对于提高生存率至关重要。