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Central Nervous System -Invading Eccrine Gland Carcinoma: A Clinicopathologic Case Series and Literature Review
- Source :
- World Neurosurgery, 138, E17-E25. ELSEVIER SCIENCE INC
- Publication Year :
- 2020
-
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.
- Subjects :
- Male
Pathology
medicine.medical_specialty
Eccrine carcinoma
Skull Neoplasms
Pembrolizumab
Eccrine Glands
Antibodies, Monoclonal, Humanized
Metastasis
03 medical and health sciences
0302 clinical medicine
Antineoplastic Agents, Immunological
medicine
Carcinoma
Humans
Neoplasm Invasiveness
Receptor, Notch1
Aged
Scalp
Spinal Neoplasms
business.industry
Brain Neoplasms
Abdominal Wall
Multimodal therapy
Middle Aged
medicine.disease
Primary tumor
Carcinoma, Ductal
Sweat Gland Neoplasms
Head and Neck Neoplasms
030220 oncology & carcinogenesis
Surgery
Female
Neurology (clinical)
business
030217 neurology & neurosurgery
Brain metastasis
Rare disease
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- World Neurosurgery, 138, E17-E25. ELSEVIER SCIENCE INC
- Accession number :
- edsair.doi.dedup.....f2686919b2f6643cdcae71c2341ff77e