AI Article Synopsis
- Eccrine carcinoma affecting the central nervous system (CNS) is rare and poorly understood in terms of treatment and prognosis, with only 23 reported cases.
- A study of 3 patients (ages 60-79) revealed varying outcomes; one had a successful treatment response with pembrolizumab due to a NOTCH1 mutation, surviving 39 months after CNS diagnosis.
- The other two patients faced rapid decline and hospice care within a month, highlighting the need for genomic testing to improve understanding and treatment strategies for this disease.
Article Abstract
Background: Eccrine carcinoma involvement of the central nervous system (CNS) is exceedingly rare. The prognosis and response to treatment of this pathology remain poorly characterized.
Methods: A retrospective case series and literature review were conducted.
Results: CNS-invading eccrine carcinoma was diagnosed in 3 patients (2 male and 1 female; age range, 60-79 years), including 2 cases of brain metastases and 1 case of brain-invading skull metastasis with subsequent spinal metastasis. The interval from primary tumor to CNS invasion was 18-51 months. All patients received multimodal therapy following diagnosis of CNS involvement. One patient who harbored a NOTCH1 mutation demonstrated a durable oncologic response after treatment with the immune checkpoint inhibitor pembrolizumab and lived 39 months after CNS invasion. The other 2 patients were discharged to hospice care within 1 month after the diagnosis of eccrine carcinoma brain metastasis. Including this case series, 23 cases of eccrine carcinoma invasion or metastasis to the CNS have been reported, with survival after diagnosis of CNS involvement ranging from a few weeks to 4 years.
Conclusions: We present 3 cases of eccrine carcinoma metastatic to the CNS, including the first reported case to our knowledge of eccrine carcinoma treated with immunotherapy. This case, harboring a NOTCH1 mutation, demonstrated the longest durable oncologic response reported in this rare disease. Genomic and molecular testing may play increasingly important roles in the evaluation of these metastases.
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| http://dx.doi.org/10.1016/j.wneu.2020.01.111 | DOI Listing |
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Article Synopsis
- Primary cutaneous mucinous carcinoma (PCMC) is a rare skin cancer that primarily develops from eccrine sweat glands, commonly appearing on the head and neck of older adults and often exhibiting slow growth.
- Although it rarely spreads to other parts of the body, PCMC has a high rate of recurrence, making early and accurate diagnosis through biopsy crucial.
- A case study of a 77-year-old man highlighted the challenges in diagnosing PCMC, leading to surgical removal and further treatment, ultimately resulting in no signs of recurrence one year later.
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