scholarly journals Merkel Cell Carcinoma of the Axilla and Adrenal Gland: A Case Report with Imaging and Pathologic Findings

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Soo Heui Baek ◽  
Hyun Kyung Jung ◽  
WooGyeong Kim ◽  
Suk Jung Kim ◽  
Hye Jin Baek ◽  
...  

Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine carcinoma of the skin. MCC is characterized by a high incidence of locoregional recurrence, and distant metastasis, and often requires short-term follow-up after treatment. In this present paper, we describe a rare case of MCC, which presented as a palpable axillary mass and an incidental adrenal mass, and report on the ultrasonography, computed tomography, and18F-fluorodeoxyglucose-positron emission tomography findings. The patient underwent surgery and adjuvant radiation therapy. Seven months after the initial diagnosis, distant metastasis was detected during a follow-up examination.

Hand ◽  
2016 ◽  
Vol 11 (4) ◽  
pp. NP24-NP29 ◽  
Author(s):  
Donevan R. Westerveld ◽  
David J. Hall ◽  
Winston T. Richards

Background: Merkel cell carcinoma (MCC) is a relatively rare and aggressive cutaneous neuroendocrine malignancy characterized by high incidence of local recurrence, distant metastases, regional nodal metastases, and high mortality. Clinically, MCC presents as a persistent asymptomatic red/pink shaped nodule, usually smaller than 2 cm with nontender intracutaneous swelling, with rapidly growing localized disease with lymph node metastases preceding distant metastases. Because of its rare nature and the lack of comprehensive understanding of the disease, management of MCC has been controversial. Methods: An 87-year-old retired Caucasian male with a history of tobacco use, chronic sun exposure, and multiple squamous and basal cell carcinomas presented with a 1.8 × 1.3 cm red, nontender nodule on the dorsum of the proximal phalanx of the left long finger first noticed 6 months prior to presentation. Biopsy was consistent with MCC after which he was treated with wide local excision, full-thickness skin grafting, and sentinel lymphadenectomy (1/4 nodes positive) followed by adjuvant radiation therapy. Results: He recovered appropriately and was clinically and radiographically disease free at 2.5-year follow-up. Conclusion: Although it remains rare, MCC has increased in incidence over the last several decades and has a predilection to occur over sun exposed areas. Highly aggressive, it has a high incidence of regional and distant metastasis as well as local recurrence. As a result, it is important that practitioners involved in the care of skin and hand lesions be aware of this condition and the need for a multidisciplinary treatment approach.


2007 ◽  
Vol 25 (9) ◽  
pp. 1043-1047 ◽  
Author(s):  
Pablo Mojica ◽  
David Smith ◽  
Joshua D.I. Ellenhorn

Purpose Merkel cell carcinoma (MCC) is a rare cutaneous malignancy. Because of the absence of randomized studies, the real benefit of adjuvant radiation therapy in MCC is unclear. The aim of this study was to better define the role of adjuvant radiation therapy in the management of MCC. Methods The Surveillance, Epidemiology, and End Results (SEER) survey from the National Cancer Institute was queried from 1973 through 2002. Retrospective analysis was performed. The end point of the study was overall survival. Results There were 1,665 cases of MCC in the SEER registry. Presentation by stage were 55% stage I, 31% stage II, and 6% stage III. Eight percent of the cases could not be staged because of incomplete data. Surgical intervention was a component of therapy in 89% of the cases (n = 1,487). The median survival for the entire cohort was 49 months, and median follow-up was 40 months. Adjuvant radiation was a component of therapy in 40% of the surgical cases. The median survival for those patients receiving adjuvant radiation therapy was 63 months compared with 45 months for those treated without adjuvant radiation. The use of radiation was associated with an improved survival for patients with all sizes of tumors, but the improvement with radiation use was particularly prominent when analyzing those patients with primary lesions larger than 2 cm. Conclusion The use of adjuvant radiation therapy is associated with improved survival in patients with MCC. Prospective evaluation of adjuvant radiation therapy in this setting is warranted.


2009 ◽  
Vol 27 (24) ◽  
pp. 4021-4026 ◽  
Author(s):  

Purpose To expedite improved understanding, diagnosis, treatment, and prevention of Merkel cell carcinoma (MCC), a rare malignancy of cutaneous neuroendocrine cells that has a 28% 2-year mortality rate. Methods This article summarizes a workshop that discussed the state-of-the-art research and priorities for research on MCC and on a new human polyomavirus (ie, MCPyV) recently discovered in 80% of MCC tumors. Results Normal Merkel cells are widely distributed in the epidermis near the end of nerve axons and may function as mechanoreceptors or chemoreceptors. Malignant MCC cells typically stain for cytokeratin 20 as well as for other epithelial and neuroendocrine markers. MCC subtypes, which are based on histology, on cell line growth properties, and on gene expression profiles, have been reported but have not been linked to prognosis. Clinical management has been empiric. MCPyV is clonally integrated at various sites in the human genome of MCC tumors, with truncating mutations in the viral, large T antigen gene that interrupt viral replication. MCPyV seroprevalence may be high, as with previously known human polyomaviruses. MCC risk is increased 11-fold with AIDS and with other cell-mediated immune deficiencies, B-cell neoplasms, and ultraviolet radiation exposure. Conclusion Development and validation of a range quantitative polymerase chain reaction and serologic assays for detection of MCPyV, as well as an infectious clone of the virus, would clarify the fundamental biology, natural history, and epidemiology of the virus, of MCC, and of other diseases. Contingent on standardized histologic diagnosis and staging of MCC, consortia are needed to clarify the risks and benefits of sentinel lymph node biopsy, adjuvant radiation therapy, and salvage therapies; consortia are needed also for epidemiologic studies of MCC etiology.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e21567-e21567
Author(s):  
Richard Cheng Han Wu ◽  
Kari Lynn Kendra ◽  
Dukagjin Blakaj ◽  
Hiral A. Shah ◽  
Joanne M. Jeter ◽  
...  

e21567 Background: Merkel Cell Carcinoma (MCC) is a cutaneous malignancy with neuroendocrine differentiation, linked to infection with polyomavirus (MCPyV) in 80% of cases. PD1 inhibitors have recently been approved for this indication with ORR, 33-56%; CR, 11-24%; PFS, about 17 months; OS, about 12 months. Nivolumab was tested in the neoadjuvant setting with similar responses with pathological CR, 47%. Methods: Adjuvant pilot study (NCT03798639) with two immunotherapy regimens administered for one year to patients with completely resected MCC at high risk of recurrence (primary lesion of 2 cm or greater, positive or close margins ( < 2 cm), perineural or lymphovascular invasion, mitotic index ≥ 20 mitotic figures per mm2, lymph node involvement (stage pIIIA or pIIIB) with or without extracapsular extension, or completely resected stage IV disease). Arm 1, nivolumab 480 mg q 4 wks and radiation therapy (RT) 50-60 Gy in 25-30 fractions, per standard of care. Arm 2, nivolumab 240 mg q 2 wks and ipilimumab 1 mg/kg q 6 wks. Primary objective was feasibility and completion of treatment in this population. Safety profile (CTCAE v5.0) and recurrence-free survival (RFS) after 18 months were secondary endpoints. Patients were randomly allocated 1:1. Results: Ten patients were screened from January 2019 until April 2020, when COVID put the study on hold and the sponsor discontinued the free drug supply. Seven were enrolled. Four were allocated to Arm 1 and three to Arm 2. Patient characteristics in Table. All patients have completed treatment and are in follow-up. Arm 1: all four patients completed radiation therapy and immunotherapy with no dose modifications or delays. Arm 2: one patient had nivolumab delayed 2 weeks for cellulitis, and another missed the last four last doses of nivolumab for cholecystitis and pancreatitis requiring surgery, unrelated to the immunotherapy. Adverse events (AE) were as expected. Arm1 caused more grade 2 and 3 AEs then Arm2 (no grade 3). One patient each discontinued treatment, in Arm 1 for progression and Arm 2 for immunotoxicity (temporal arteritis grade 2). One recurrence was observed in Arm 1 and none in Arm 2. Conclusions: The number of patients expected to recur at 1 year is 20%. Our observed data is insufficient to establish efficacy. However with no patient recurring in the ipilimumab arm after 18 months of follow-up and lower observed side effects, we would favor this regimen for the next trial. Clinical trial information: NCT03798639. [Table: see text]


2020 ◽  
Vol 16 (16) ◽  
pp. 1101-1113 ◽  
Author(s):  
Yi-Jun Xia ◽  
Dong-Sheng Cao ◽  
Jun Zhao ◽  
Bang-Zhong Zhu ◽  
Juan Xie

Aim: To describe the factors affecting distant metastasis of Merkel cell carcinoma (MCC) and the prognosis of metastatic MCC. Materials & methods: The MCC patient information was downloaded from the SEER database. Logistic regression and Cox proportional hazard models were conducted to screen for significant factors. Results: A total of 3449 patients were enrolled. Surgery and chemotherapy were significantly correlated with the occurrence of distant metastasis. In the cause-specific survival rate of MCC, regional lymph node removal, sentinel lymph node biopsy, radiation and chemotherapy can significantly reduce the prognostic risk of patients with distant metastases. Conclusion: Our study screened out the factors affecting the distant metastasis and prognosis of MCC and more prospective studies are needed to verify our findings.


2019 ◽  
Vol 103 (5) ◽  
pp. E47-E48
Author(s):  
Praveen Pendyala ◽  
John Byun ◽  
Sharad Goyal ◽  
James Goydos ◽  
Ann W. Silk ◽  
...  

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]


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