Merkel Cell Carcinoma Presenting as Subcutaneous Breast Masses: An Uncommon Presentation of a Rare Neuroendocrine Neoplasm

2015 ◽  
Vol 22 (1) ◽  
pp. 113-115 ◽  
Author(s):  
Vinod E. Nambudiri ◽  
Marina Vivero ◽  
Alice J. Watson ◽  
Manisha Thakuria ◽  
Andrea Ng ◽  
...  
2019 ◽  
Vol 2019 (11) ◽  
Author(s):  
Nicholas Mulchan ◽  
Alberto Cayton ◽  
Armand Asarian ◽  
Philip Xiao

Abstract Merkel cell carcinoma (MCC) is an aggressive cutaneous malignancy of neuroendocrine origin presenting as a painless, rapidly growing nodule. MCC often presents in elderly, fair-skinned individuals in sun-exposed areas. Diagnosis is often overlooked at time of presentation due to its rarity, but MCC is twice as deadly as malignant melanoma. There has been bigger interest in the disease due to increasing incidence and an association with the prevalent virus Merkel cell polyomavirus. This study describes an uncommon presentation of MCC as a right gluteal lesion in an Afro-Panamanian patient. The tumor was suspected to be fibrolipoma, but Immunohistochemistry revealed the diagnosis of MCC, as stains for CD56 and CK20 were positive. In addition to surgical excision, the patient was referred for adjuvant radiotherapy. This case report and literature review elucidates the clinical, histopathologic and management aspects of MCC, which will help in recognizing and treating these tumors.


2020 ◽  
Vol 38 (22) ◽  
pp. 2471-2475
Author(s):  
Andrew S. Brohl ◽  
Vernon K. Sondak

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 78-year-old man presented with rapidly enlarging lymph nodes in the right preauricular region and neck. Needle biopsy revealed a cytokeratin 20–positive, high-grade neuroendocrine neoplasm consistent with Merkel cell carcinoma (MCC). Cross-sectional imaging disclosed a 5.2-cm intraparotid mass and extensive adenopathy in the ipsilateral cervical and submental chains ( Figs 1A and 1C ), without distant metastatic disease. A skin examination did not reveal a primary lesion (hence, stage IIIA, T0N1bM0). The patient’s history was notable for hypertension, diet-controlled diabetes type II, high cholesterol, and a past history of numerous cutaneous basal and squamous cell carcinomas. He was quite active but reported discomfort from the bulk of the tumors. The patient was evaluated by the surgical oncology team, who believed that the parotid mass and cervical adenopathy were technically resectable but that resection carried a substantial risk of morbidity because of the potential need to sacrifice the facial and/or spinal accessory nerves and because of a likely margin-positive (R1 or R2) result. He was referred to the medical oncology team to discuss management options for regionally advanced, “borderline-resectable” MCC.


2003 ◽  
Vol 89 (2) ◽  
pp. 146-151 ◽  
Author(s):  
Fernando Cirillo ◽  
Massimo Buononato ◽  
Gianfranco Lima ◽  
Ines Cafaro ◽  
Palmiro Alquati

Merkel cell carcinoma is a rare neuroendocrine neoplasm of the skin. The tumor most frequently affects elderly patients, with a preference for the head and neck. Eight patients affected by Merkel cell carcinoma have been observed at the General Surgery Unit II of the “Istituti Ospitalieri” hospital in Cremona, each in different stages of the disease; 75% of the cases involved the extremities, and in nearly all of the cases the tumor was nodular in appearance, with an average diameter of 2.2 cm. In 2 cases, the tumor was associated with rheumatoid arthritis, suggesting a dependency on the part of the neoplasm on the immune disorder and on steroid treatment. The available data confirm that in stage I of the disease, surgical treatment should be associated with radiotherapy in order to control the development of local relapses or metastases over time. In this stage, we observed a survival of 34 months (range, 24-48). In stages II and III, survival time falls, with very short duration of responses and poor quality of life as a result of the administration of cytotoxic molecules. Bearing in mind that any local relapse tends to appear within 12 months of the removal of the primitive tumor, that lymph node metastases appear in almost half of the patients, and that metastases over time are manifested in over a third of patients, it is essential to adopt a treatment capable of balancing the demand for longer remissions with a better quality of life. In this situation, we observed that treatment with somatostatin analogues achieves interesting responses without side effects, which suggests a close biological relationship between the tumor and somatostatin and that making a careful assessment of the prognostic factors of the disease can guarantee a correct therapeutic choice.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Daniele Rossini ◽  
Salvatore Caponnetto ◽  
Vittoria Lapadula ◽  
Lucilla De Filippis ◽  
Gabriella Del Bene ◽  
...  

Merkel cell carcinoma (MCC) is an extremely rare primary neuroendocrine neoplasm of the skin that shows aggressive behavior and a poor prognosis. We report a case of a 67-year-old male with a Merkel cell carcinoma which initially presented itself as a large retroperitoneal mass. Pathological and immunohistochemical analysis revealed tissue consistent with neuroendocrine carcinoma. Despite complete medical workup, no other primary MCC could be detected. While being an atypical presentation, the tumor mass showed an excellent response to the combination of chemotherapy followed by radiotherapy.


Author(s):  
Ara Cho ◽  
Mi Jang ◽  
In Seok Moon ◽  
Seung Ho Shin

Merkel cell carcinoma is an extremely rare neuroendocrine neoplasm of the external auditory canal (EAC) skin, which requires wide excision and adjuvant radiation due to a high recurrence rate. In this report, we describe a case of Merkel cell carcinoma arising from the EAC which was successfully treated with endoscopic excision. A 32-years-old female patient with a history of papillary thyroid cancer was diagnosed with an EAC tumor incidentally. There was a erythematous papular lesion on the posterior EAC without any evidence of locoregional metastasis. The patient underwent endoscopic tumor resection under general anesthesia, and the final pathologic report confirmed the diagnosis of Merkel cell carcinoma. There was no clinical or radiographic evidence of recurrence or metastasis of Merkel cell tumor for 41 months after surgical resection alone.


2016 ◽  
Vol 3 (1) ◽  
pp. 32
Author(s):  
Hanane Atarguine ◽  
Said Amal

Merkel cell carcinoma remains a rare cutaneous neuroendocrine malignancy that exhibits clinically aggressive features and is associated with a poor prognosis.


Cancers ◽  
2018 ◽  
Vol 10 (10) ◽  
pp. 350 ◽  
Author(s):  
Marco Rastrelli ◽  
Beatrice Ferrazzi ◽  
Francesco Cavallin ◽  
Vanna Chiarion Sileni ◽  
Jacopo Pigozzo ◽  
...  

Merkel Cell Carcinoma (MCC) is a rare but highly aggressive neuroendocrine neoplasm of the skin. This study aimed at describing characteristics, treatment, and prognosis of a series of consecutive cases of MCC patients, in order to contribute to the investigation of this rare malignancy and provide better patient care. This is a retrospective cohort study including all 90 patients diagnosed and/or treated for MCC between 1991 and 2018 at the Veneto Institute of Oncology in Padua (Italy). Patient and tumor characteristics, treatment, and immunohistochemical data were extracted from a prospectively collected local database. There were 68 primary (76%) and 22 non-primary (15 occult primary, three metastatic, four recurrence) tumors (24%). CK20 expression was associated with reduced overall (HR 2.92, 95% CI 1.04–8.16) and disease-specific (HR 4.62, 95% CI 1.31–16.28) survival. Immunomodulatory regimens for treatment of other comorbidities were associated with reduced disease-specific ((HR 2.15, 95% CI 1.06–4.36) and recurrence-free (HR 3.08, 95% CI 1.44–6.57) survival. Iatrogenic immunomodulation resulted as the main factor associated with impaired prognosis. Lack of CK20 expression was associated with better survival.


2021 ◽  
Author(s):  
Vittorio Durastante ◽  
Antonello Conte ◽  
Pier Paolo Brollo ◽  
Carlo Biddau ◽  
Michele Graziano ◽  
...  

Abstract Introduction. Merkel cell carcinoma (MCC) is an infrequent, but highly aggressive neuroendocrine neoplasm of the skin with a propensity for recurrence and metastasis. We report a rare case of gastric metastatic localization of this cancer by focusing on the diagnostic, clinical and surgical approach to the patient. Case report. Clinical presentation begins with a peripheral lymphadenopathy whose immunohistochemical characterization identifies the lymphatic dissemination of the disease. Gradually the patient develops a severe anaemic state which requires several blood transfusions and surgical gastric resection to remove a large bleeding lesion of the antral region. The histopathological analysis of the specimen confirms the metastatic origin from MCC, but the primitive lesion remains unknown. Discussion. Since this clinical situation is very rare, we conducted a review of the literature selecting the few cases reported, in order to evaluate the current knowledge on this topic. Metastatic involvement of the stomach from Merkel cell carcinoma is a rare presentation of this disease progression with a frequent delay in formulating the correct diagnosis and in further treatment which may be life-threatening for the patient. As regards the local treatment, there is no specific guideline, and the therapeutic indication should be tailored on the specific case.


2012 ◽  
Vol 16 (1) ◽  
pp. 54-60 ◽  
Author(s):  
Brian Seaman ◽  
Steven Brem ◽  
Allison Fromm ◽  
Aileen Staller ◽  
Timothy Mccardle ◽  
...  

Background: Merkel cell carcinoma (MCC) is a rare, highly malignant, and aggressive dermal neuroendocrine neoplasm that rarely metastasizes to the central nervous system. Objective: To review the current literature regarding treatment of neurometastatic MCC. Methods: A case of a 78-year-old male with intracranial extra-axial metastatic MCC involving the left cerebellopontine angle is presented. Results: A retrosigmoid craniectomy was performed with complete resection of the metastatic focus. Adjuvant treatment included whole-brain radation therapy followed by etoposide and carboplatin chemotherapy. Seven months postoperatively, the patient was free of metastatic disease. Conclusion: Surgical resection should be performed when feasible to prevent local recurrence. This may be followed by early adjuvant fractionated whole-brain radiotherapy and systemic chemotherapy; however, no clinical trials have been performed to demonstrate a survival benefit.


2015 ◽  
Vol 81 (8) ◽  
pp. 802-806 ◽  
Author(s):  
Timothy L. Fitzgerald ◽  
Samuel Dennis ◽  
Swapnil D. Kachare ◽  
Nasreen A. Vohra ◽  
Jan H. Wong ◽  
...  

Merkel cell carcinoma is a cutaneous neuroendocrine neoplasm that has been poorly studied in contemporary cohorts. Patients with Merkel cell carcinoma from 1986 to 2011 were identified in the Surveillance Epidemiology and End Results registry. A total of 5211 patients met the inclusion criteria. The mean age was 74.9 years; majority were male (61.4%) and white (94.9%). Patients were divided into two cohorts: Group 1 (1986 and 1999) and Group 2 (1999–2010). Group 2 was more likely to have Stage III disease (14.6 vs 23.3%, P < 0.001) and less likely to have Stage I/II disease (71.8 vs 65.1%, P < 0.0001). The increase in Stage III was likely secondary to increased use of sentinel lymph node biopsy. Disease-specific five-year survival for Stages I/II was 78.1 per cent and Stage III was 54 per cent. Disease-specific five-year survival was unchanged between Groups 1 and 2, 69.9 versus 66.6 per cent, respectively ( P = 0.44). Both incidence and mortality significantly increased over the study period with P value for both trends <0.0001. In 1986, incidence and mortality rates per 100,000 were 0.22 and 0.03, respectively, and increased to 0.79 and 0.43 in 2011, respectively. There has been a greater than 333 per cent increase in mortality from Merkel cell carcinoma.


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