Direct intracranial invasion of eccrine spiradenocarcinoma of the scalp: a case report and literature review

Abstract

Background Eccrine spiradenocarcinoma (SC), also known as malignant eccrine spiradenoma, is a rare malignant cutaneous adnexal neoplasm arising from long-standing benign eccrine spiradenoma. Malignant skin tumors rarely show direct intracranial invasion. However, once the intracranial structure is infiltrated, curative excision with sufficient margins can become extremely difficult, particularly when the venous sinuses are involved. No effective adjuvant therapies have yet been established. Here, we report an extremely rare case of scalp eccrine SC with direct intracranial invasion, which does not appear to have been reported previously. Case presentation An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC. Conclusions Scalp SC with direct intracranial invasion is extremely rare. Radical resection with tumor-free margins is the mainstay of treatment, but the involvement of venous sinuses makes this unfeasible. High-precision EBRT in combination with maximal resection preserving the venous sinuses could be a treatment option for local tumor control.

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

“…2,3,6,11 A recently reported case of a low-grade spiradenocarcinoma of the scalp led to eventual death after intracerebral extension of a recurrent tumor, without metastatic spread. 7 To the best of our knowledge there are only two additional reports of morphologically low-grade spiradenocarcinoma resulting in distant metastasis. 8,15 In the first of these cases, the patient was a young 29-year-old male who developed lung metastases and died of disease 11 years after the initial presentation.…”

“…While the morphologically high‐grade tumors may behave aggressively with risk for distant metastatic spread and mortality, the morphologically low‐grade spiradenocarcinomas appear to be largely indolent with risk for local recurrence of around 19% but no reported distant metastasis or mortality in larger cohorts 2,3,6,11 . A recently reported case of a low‐grade spiradenocarcinoma of the scalp led to eventual death after intracerebral extension of a recurrent tumor, without metastatic spread 7 …”

“…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.…”

“…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 Eccrine Spiradenoma of the Posterior Scalp: An Odd Presentation

“…Despite not being typically utilized as initial therapy, radiotherapy has been used for palliative care [22], as well as to treat positive margins and gross residual tumors [23][24][25][26][27]. In one reported case, the patient diagnosed with MES of the thigh with regional lymph node metastasis was treated with radical surgical excision with lymph node dissection followed by a total dose of 50 Gy to both the tumor bed and inguinal lymph nodes using external beam radiotherapy (EBRT), though fractionation was not reported [26].…”