Challenges in diagnosis and management of a spiradenocarcinoma: a comprehensive literature review

Abstract

Background: Spiradenocarcinoma is a rare skin adnexal neoplasm that may behave aggressively. It is often associated with a benign slow-growing spiradenoma that has undergone malignant transformation. Given the paucity of cases in the literature, there is a lack of consensus on treatment. Methods: The terms 'malignant spiradenoma' or 'spiradenocarcinoma' were systematically used to search the PubMed, MEDLINE and Google Scholar databases. A total of 182 cases of spiradenocarcinoma were identified as eligible for this comprehensive literature review. Results: Spiradenocarcinoma was commoner in older age and Caucasian race. In most cases, surgical excision for local disease is the mainstay of treatment. Lymph node dissection is usually reserved for those with suspected or confirmed lymph node metastases. High rates of local recurrence (20.8%), metastasis (37.4%) and mortality (19.1%) were identified, prompting some authors to suggest regular follow up including chest X-rays and liver function tests. Conclusions: Patients with spiradenocarcinoma may benefit from a magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography/computed tomography to establish the extent of disease. We recommend wide local excision as the treatment of choice to achieve surgical margins of ≥1 cm, with node resection to be determined on a case-to-case basis. Regular follow up is important given the high rate of local recurrence, metastasis and mortality. This should include an examination of the regional lymph nodes. Further research is required to refine an evidence-based approach to spiradenocarcinoma.

A malignant spiradenoma of the forehead: a case report and literature review

“…MSA is a rare adnexal tumor that could pose both a diagnostic and a therapeutic challenge due to the paucity of published data regarding this entity. To date, there are no consensus nor clear guidelines for the management of MSA, and therapeutic options are obtained from published case reports or case series [ 8 ]. The incidence of this rare entity was estimated to be 0.07 cases per million.…”

“…The tumor would classically present as an abrupt growth of a long-standing indolent, painless nodule. Sometimes, pain and ulceration are associated features, which could be the main driver for seeking medical advice [ 8 ]. The time between appearance of the benign lesion and malignant transformation is variable, ranging from 6 months to 70 years, but it is usually around 20–30 years on average [ 9 ].…”

Challenges in the diagnosis and treatment of the malignant adnexal neoplasms of the head and neck

“…Several markers reported to label dermal progenitor cells are preserved in the MANs, including the tumor stroma (CRABP1, Nestin, and Ephrin B2) [31]. Some authors recommend magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography/ computed tomography to establish the extent of disease [32]. Moreover, new biomarkers with high diagnostic value related to specific molecular alterations demonstrated in MANs have been discovered recently (Table 4).…”

Distant metastasis from morphologically low‐grade spiradenocarcinoma: A report of two cases

“…Malignant neoplasms arising from spiradenoma, cylindroma, and spiradenocylindroma are rare malignant sweat gland tumors, the majority of which arise in association with a benign spiradenoma precursor (spiradenocarcinoma) 1–15 . Their histopathologic separation into morphologically low‐ and high‐grade spiradenocarcinomas appears to correlate well with clinical behavior and outcome 2,3,6,11 .…”

“…Malignant neoplasms arising from spiradenoma, cylindroma, and spiradenocylindroma are rare malignant sweat gland tumors, the majority of which arise in association with a benign spiradenoma precursor (spiradenocarcinoma). [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Their histopathologic separation into morphologically low-and high-grade spiradenocarcinomas appears to correlate well with clinical behavior and outcome. 2,3,6,11 While the morphologically high-grade spiradenocarcinomas may be associated with aggressive behavior, including distant metastases and mortality, the morphologically low-grade tumors appear to be indolent, mainly showing increased risk for local recurrence only.…”