Merkel cell carcinoma of an unknown primary

2020 ◽  
Vol 18 (3) ◽  
pp. 253-255
Author(s):  
Drucilla Edmonston ◽  
Lillie O’steen ◽  
Kristianna Fredenburg ◽  
William M. Mendenhall
2019 ◽  
Vol 46 (8) ◽  
Author(s):  
Shiho Hanai ◽  
Takatoshi Shimauchi ◽  
Reiko Kageyama ◽  
Masahiro Aoshima ◽  
Taisuke Ito ◽  
...  

2014 ◽  
Vol 27 (9) ◽  
pp. 1182-1192 ◽  
Author(s):  
Zenggang Pan ◽  
Yuan-Yuan Chen ◽  
Xiaojun Wu ◽  
Vijay Trisal ◽  
Sharon P Wilczynski ◽  
...  

2020 ◽  
pp. 030089162094420
Author(s):  
Dario Zerini ◽  
Filippo Patti ◽  
Francesca Spada ◽  
Nicola Fazio ◽  
Eleonora Pisa ◽  
...  

Objective: To review the therapeutic strategy in Merkel cell carcinoma (MCC) treated with radiotherapy (RT) discussed in a multidisciplinary tumour board. Methods: Clinical records of patients with a diagnosis of MCC and with an indication to undergo RT at the European Institute of Oncology between 2003 and 2018 were reviewed retrospectively. Results: Twenty-six patients were included in the analysis (median age 65 years, range 42–87). Nineteen received adjuvant RT, 4 exclusive RT, and the remainder palliative RT. Intensity-modulated RT was used in 13 cases, a 3D conformal technique in 11 cases, and stereotactic RT in 2 cases. No major toxicities were recorded. The median relapse-free survival (RFS) after adjuvant RT was 20.5 months, while for unknown primary MCC, it was 23 months. In the adjuvant setting, median polyomavirus-positive RFS was 21.5 months (range 1–49) and median polyomavirus-negative RFS was only 14 months (range 4–45). Overall, RFS of polyomavirus-positive and polyomavirus-negative patients was 10.5 and 8 months, respectively. After adjuvant RT, only 1 out of 10 patients had a recurrence in the RT field. At the time of data collection, 16 patients were alive with no evidence of disease, 1 patient was alive with advanced status of disease, 8 patients died of disease progression, and 1 patient died of other causes. Conclusions: The management of unknown primary and polyomavirus-positive cases, which had a better prognosis in our series, may benefit from a multidisciplinary approach, given the limited data available regarding optimal treatment.


Author(s):  
Y Nazarian ◽  
B Shalmon ◽  
Z Horowitz ◽  
L Bedrin ◽  
M R Pfeffer ◽  
...  

Merkel cell carcinoma is an uncommon and aggressive primary neuroendocrine skin malignancy which mostly affects the extremities and the head and neck region of elderly patients. Merkel cell carcinoma occurs with increased frequency in sun-exposed areas, in individuals exposed to arsenic and in immunosuppressed patients. Many patients with Merkel cell carcinoma present with other malignancies, mainly skin cancers. Characteristic features are frequent recurrences and regional and distant metastases. Mortality rates range from 20 to 65 per cent. The mainstay of treatment is surgery, with wide local excision, and adjuvant radiotherapy is usually administered. Merkel cell carcinoma of unknown primary site is rare, and the majority of the few cases described have not been from head and neck areas. We present a case of Merkel cell carcinoma of unknown primary site, with upper neck and distant metastases.


2019 ◽  
Vol 12 (2) ◽  
pp. e224834
Author(s):  
João Vasco Barreira ◽  
Margarida Moura Valejo Coelho ◽  
Catarina Ribeiro ◽  
Mónica Semedo

The authors present the case of a woman in the seventh decade of life with medical history of: left nephrectomy for renal tuberculosis and non-Hodgkin’s lymphoma treated with chemotherapy (QT) and radiotherapy. She presented with a 2-month history of non-tender, left inguinal lymph node enlargement. Positron Emission Tomography (PET)—CT —scanshowed hypermetabolic inguinal and retroperitoneal lymphadenopathies, no primary tumour. On the second dermatological examination a pink, 2 cm plaque on the anterior left knee was noted. The histopathological analysis revealed Merkel cell carcinoma. The patient underwent two lines of systemic QT, with life-threatening toxicities limiting treatment. Followed overwhelming disease progression with lymphoedema and numerous skin metastases in the left lower limb. The patient received palliative care until death. The rare incidence of such neoplasia and its uncommon clinical presentation justifies reporting this case and highlights the importance of multidisciplinary teams in the management of cancer patients.


2020 ◽  
Vol 154 (3) ◽  
pp. 369-380
Author(s):  
Lauren E B Lawrence ◽  
Atif Saleem ◽  
Malaya K Sahoo ◽  
Susanna K Tan ◽  
Benjamin A Pinsky ◽  
...  

Abstract Objectives The possibility of a so-called primary lymph node neuroendocrine carcinoma has been described in the literature. Here we evaluate cases fitting such a diagnosis and find that the cases demonstrate a convincing and pervasive pattern consistent with metastatic Merkel cell carcinoma. Methods Six cases of primary lymph node Merkel cell carcinoma and one case of metastatic neuroendocrine carcinoma at a bony site, all with unknown primary, were sequenced using a combination of whole-exome and targeted panel methods. Sequencing results were analyzed for the presence of an ultraviolet (UV) mutational signature or off-target detection of Merkel cell polyomavirus (MCPyV). Results Four of six primary lymph node cases were positive for a UV mutational signature, with the remaining two cases positive for off-target alignment of MCPyV. One case of neuroendocrine carcinoma occurring at a bony site was also positive for a UV mutational signature. Conclusions We find no evidence to corroborate the existence of so-called primary Merkel cell carcinoma of lymph node.


2018 ◽  
Vol 113 (Supplement) ◽  
pp. S1318
Author(s):  
Shravani Reddy ◽  
Meeta R. Desai ◽  
Joshua Eikenberg ◽  
Vikas Chitnavis ◽  
Douglas Grider

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).


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