Martin Shabiah, DeJesus Jana, Jacob Ann, Qvavadze Teah, Guerrieri Claudio, Hudacko Rachel, Boucree Thaddeus
Newark Beth Israel Medical Center, 201 Lyons Ave, Newark, NJ 07112, USA.
Int J Surg Case Rep. 2019;65:284-287. doi: 10.1016/j.ijscr.2019.10.087. Epub 2019 Nov 6.
Pilomatrix carcinoma is a rare aggressive tumor with a high rate of local recurrence after surgical excision. Diagnosis is made by histopathology and when discovered, wide local excision has been shown to have the best results.
We report a case of a 74-year-old male incidentally found to have a large right postauricular mass and regional lymphadenopathy. The mass was biopsied and proven to be a malignant pilomatrixoma. Wide local excision and level II and III neck dissection with reconstruction using a right supraclavicular flap was performed.
Pilomatrix carcinoma is a lesion first described in 1880 by Malherbe and Chenantais. It is unknown if these tumors arise de novo or arise through malignant transformation of a benign pilomatrixoma. There are similarities between the benign lesion and its malignant counterpart in terms of activating mutations in signaling pathways. A well-defined gold standard for surgical management has not been established, but currently wide local excision with safe margins is recommended along with regional lymph node dissection when metastasis is suspected. Currently, no chemotherapy regimen has been shown to be effective in local control or in preventing metastatic spread.
Pilomatrix carcinoma, given its aggressive nature, has a high propensity for recurrence after excision. It is important to perform wide local excision to avoid an incomplete resection and higher recurrence rates. Further studies will be needed to create a more defined standard of treatment and to evaluate the role of adjuvant chemotherapy and radiation therapy.
毛发基质癌是一种罕见的侵袭性肿瘤，手术切除后局部复发率高。通过组织病理学进行诊断，一旦发现，广泛局部切除已被证明效果最佳。
我们报告一例74岁男性，偶然发现右侧耳后有一个大肿块及区域淋巴结肿大。对肿块进行活检，证实为恶性毛发基质瘤。进行了广泛局部切除以及II级和III级颈部清扫，并使用右锁骨上皮瓣进行重建。
毛发基质癌是1880年由马尔赫布和舍南泰首次描述的病变。这些肿瘤是原发产生还是由良性毛发基质瘤恶变而来尚不清楚。在信号通路的激活突变方面，良性病变与其恶性对应物之间存在相似之处。尚未确立明确的手术治疗金标准，但目前建议进行切缘安全的广泛局部切除，当怀疑有转移时还应进行区域淋巴结清扫。目前，尚无化疗方案被证明对局部控制或预防转移扩散有效。
鉴于其侵袭性，毛发基质癌切除后复发倾向高。进行广泛局部切除以避免切除不完全和更高的复发率很重要。需要进一步研究以制定更明确的治疗标准，并评估辅助化疗和放疗的作用。
