scholarly journals Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Sophia Z. Shalhout ◽  
Kevin S. Emerick ◽  
Peter M. Sadow ◽  
Jenny J. Linnoila ◽  
David M. Miller

Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine cancer with a high risk of recurrence and metastasis. MCC is generally associated with advanced age, fair skin, sun exposure, immunosuppression, and in the majority of cases, the Merkel cell polyomavirus. Neuroendocrine malignancies are associated with a variety of paraneoplastic neurological syndromes (PNS), characterized as autoimmune responses to malignancy-associated expression of neural antigens. Our literature review underscores previous case reports of MCC-associated PNS with voltage-gated calcium channel (VGCC) and anti-Hu (or ANNA-1) autoantibodies. We present the case of a 59-year-old male with regionally metastatic Merkel cell carcinoma complicated by the paraneoplastic manifestation of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. His primary lower neck subcutaneous MCC and metastasis were initially treated with surgery. Additional recurrent lymph node metastases were successfully treated with definitive intensity-modulated radiation therapy. His PNS improved with rituximab therapy. Although rare, this case highlights that in the setting of seizures and prominent psychiatric symptoms accompanying an MCC diagnosis, evaluation for autoimmune paraneoplastic encephalitis is warranted. Awareness and detection of preexisting PNS are crucial in the era of immune checkpoint inhibitors (ICI) for advanced MCC, where treatment with ICI has the potential to exacerbate preexisting autoimmune PNS and lead to worsened or even lethal neurologic immune-related adverse events (nirAEs).

2020 ◽  
Vol 4 (6) ◽  
pp. 559-562
Author(s):  
Margaret Coleman Maxi ◽  
Joel Byrne ◽  
Ardenne Martin ◽  
Pamela Martin

Merkel Cell Carcinoma (MCC) is a rare and aggressive neuroendocrine carcinoma that most commonly occurs in elderly Caucasian males who have a history of extensive sun exposure. Most cases of Merkel Cell Carcinoma (MCC) present in the outpatient setting and include a single, asymptomatic, red or pink lesion on sun exposed areas with a diameter less than 2 cm. There was a threefold increase in the incidence of MCC from 1986 to 2001, and the incidence of MCC is predicted to continue to increase secondary to the aging “baby boomer” population.To our knowledge, there are only two case reports that describe MCC resembling and receiving the diagnosis of cellulitis at initial presentation. We report an unusual case of MCC presenting as suspected cellulitis of the chest wall in an elderly Caucasian male.  


ISRN Surgery ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-17 ◽  
Author(s):  
Patricia Tai

The role of surgeons in the treatment of Merkel cell carcinoma (MCC) of the skin is reviewed, with respect to diagnosis and treatment. Most of the data in the literature are case reports. Surgery is the mainstay of treatment. A wide local excision, with sentinel node (SLN) biopsy, is the recommended treatment of choice. If SLN is involved, nodal dissection should be performed; unless patient is unfit, then regional radiotherapy can be given. Surgeons should always refer patients for assessment of the need for adjuvant treatments. Adjuvant radiotherapy is well tolerated and effective to minimize recurrence. Adjuvant chemotherapy may be considered for selected node-positive patients, as per National Comprehensive Cancer Network guideline. Data are insufficient to assess whether adjuvant chemotherapy improves survival. Recurrent disease should be treated by complete surgical resection if possible, followed by radiotherapy and possibly chemotherapy. Generally results of multimodality treatment for recurrent disease are better than lesser treatments. Future research should focus on newer chemotherapy and molecular targeted agents in the adjuvant setting and for gross disease.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e15696-e15696
Author(s):  
Aman Chauhan ◽  
Leaundra Murray ◽  
Arun Kumar Arumugam Raajasekar ◽  
Zin Myint ◽  
Lowell Brian Anthony

e15696 Background: Neuroendocrine tumor of unknown primary constitutes about 10-15 % of all neuroendocrine tumors. Identification of primary site can helps alter the management. Sunitinib is FDA approved for management of pancreatic neuroendocrine tumors, everolimus is approved for gastroenteropancreatic and bronchial NETs, immune checkpoint inhibitors are active in Merkel cell carcinoma and MIBG treatment is standard of care for pheochromocytoma. Methods: Patients with neuroendocrine tumor with unknown primary were identified from Markey Cancer Center database over a five-year period (2012-2016). Patient who underwent 92-gene reverse transcriptase polymerase chain reaction cancer classification assay (BioTheranostics Tissue Type ID) were analyzed. IRB approval was obtained. Results: 56 patients with neuroendocrine tumors with unknown primary were identified. Median age of cohort was 61 years. 28/56 patients were males. 92 gene cancer ID assay was used in 38 out of 56 patients. Primary site of tumor was identified with > 95% certainty in 36 out of 38 patients. The test reported pancreatic NET as the primary site for 10 patients, gastrointestinal NETs for 14 patients, bronchial carcinoid for 5, large call NEC for 3, Merkel cell carcinoma for two and pheochromocytoma in one patient. Conclusions: Tissue type ID was able to identify a primary site in NETs of unknown primary in majority (94.7%) of cases. The result had direct implication in management of patients with regards to FDA approved treatment options in 13/38 patients (pNETs, merkel cell and pheochromocytoma).


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e14098-e14098
Author(s):  
Theresa N Canavan ◽  
Nicole Adell Doudican ◽  
Mary Stevenson ◽  
Anna C. Pavlick ◽  
John Carucci

e14098 Background: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine carcinoma of the skin that is highly immunogenic. Checkpoint inhibitors (CPI) have recently revolutionized the treatment of advanced MCC. In this study we sought to better understand how CPI are used in an outpatient setting and to better define MCC outcomes associated with their use. Methods: We conducted a retrospective chart review of MCC patients seen in the New York University Hematology and Oncology Department from 2012-2018. Patient characteristics and treatment regimens were compared between those with and without disease progression at any point during follow-up. Results: Fifteen patients were identified, 46.7% of whom presented with nodal or distant disease (Table). Nine patients experienced relapse during follow-up. There were no MCC-specific deaths, and 92.3% of patients were without evidence of MCC at the end of follow-up. Ten patients were treated with one or more CPI (pembrolizumab, nivolumab, ipilimumab) either in the setting of first line systemic therapy (71.4%) or after experiencing disease progression (28.6%). There was a trend toward improved relapse free survival with CPI use (P = 0.054). Conclusions: Although recurrences were common, overall outcomes at the end of follow-up were very good. CPI were well tolerated and were associated with a trend toward improved relapse free survival. Patients with advanced stage MCC would benefit from consideration of CPI as part of their treatment options. [Table: see text]


2016 ◽  
Vol 143 (2) ◽  
pp. 347-350 ◽  
Author(s):  
T. Gambichler ◽  
U. Wieland ◽  
S. Silling ◽  
M. Dreißigacker ◽  
J. Schaller ◽  
...  

2017 ◽  
Vol 6 (3) ◽  
pp. 205846011770044 ◽  
Author(s):  
Maria Kouzmina ◽  
Virve Koljonen ◽  
Junnu Leikola ◽  
Tom Böhling ◽  
Eila Lantto

Background The primary neuroendocrine skin cancer, Merkel cell carcinoma (MCC), has a well-known predilection to metastasize systemically. However, the experience of systemic metastases in MCC is mainly disseminated through case reports due to the rarity of MCC. Purpose To elucidate the frequency and locations of systemic metastasis in MCC by reviewing the imaging of patients with metastatic MCC in a national cohort. Material and Methods Patients with diagnosed metastatic MCC by imaging studies in Finland during 1999–2012 were included in this study. We reviewed their imaging studies to evaluate the most frequent sites for systemic metastasis and determined the latency between the primary tumor diagnosis and systemic metastasis. The material includes 30 MCC patients with complete imaging series and 187 examinations, of which 102 (54%) were CT images. Results The mean latency from the primary tumor diagnosis to systemic metastasis was 2.1 years and the mean latency between the radiologic diagnosis of the metastases and death was 299 days. Metastases were recorded in several organ systems in most of the cases, and at least two separate metastatic sites in 63% of the cases. Metastatic spread was noted in 60% of the cases in distant lymph nodes. Liver and lungs were the most affected solid organs. Conclusion Systemic metastasis in MCC has no predilection site, basically every organ system can be involved. Most of the systemic metastases were recorded during the first two years after the MCC diagnosis.


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