scholarly journals Prognostic Factors in Merkel Cell Carcinoma: A Retrospective Single-Center Study in 90 Patients

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.

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.


2019 ◽  
Vol 28 (3) ◽  
pp. 325-329
Author(s):  
Sean Hacking ◽  
Hector Chavaria ◽  
Leonard Khan ◽  
Kasturi Das

Merkel cell carcinoma (MCC) is a rare entity that most commonly arises from the skin. Angiosarcoma (AS) is a rare malignancy with a predilection for elderly males, has endothelial differentiation and a notoriously poor prognosis despite aggressive therapy. Herein, we report an angiosarcoma colliding with a MCC, in a patient with a past medical history of squamous cell carcinoma, status-post radiation therapy. More specifically, our case represents a collision tumor, a rare entity composed of two histologically distinct neoplasms which coincide together at the same location. This case represents the first documented report of such a presentation. With that being said, its clinical course, prognosis, pathogenesis, and molecular profile, currently remains unclear. Importantly, neoplasms are increasingly being found to be associated with radiation therapy, of which our patient had received. Ultimately, however, with the lack of c-MYC immunohistochemical staining, and a short duration between radiation exposure and presentation, the AS in our case was likely coincidental.


2015 ◽  
Vol 22 (1) ◽  
pp. 113-115 ◽  
Author(s):  
Vinod E. Nambudiri ◽  
Marina Vivero ◽  
Alice J. Watson ◽  
Manisha Thakuria ◽  
Andrea Ng ◽  
...  

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.


2021 ◽  
pp. 229255032110319
Author(s):  
Frances-Claire Eichorn ◽  
Arbind Dubey ◽  
Kumar Alok Pathak

Merkel cell carcinoma (MCC) of the head and neck is a rare and aggressive non-melanoma skin cancer. The objective of this study was to assess the oncological outcome of MCC by retrospective review of electronic and paper records of a population-based cohort of 17 consecutive cases of the head and neck MCC without distant metastasis, diagnosed in Manitoba between 2004 and 2016. The average age of the patients at initial presentation was 74.1 ± 14.4 years with 6 patients presenting with stage I, 4 with stage II, and 7 with stage III disease. Both surgery or radiotherapy alone were the primary treatment modalities in 4 patients each and the remaining 9 patients had a combination of surgery with adjuvant radiotherapy. During the median follow-up of 52 months, 8 patients had recurrent/residual disease and 7 eventually died of it ( P = .001). Metastatic spread of disease to the regional lymph nodes was observed in 11 patients either at presentation or during the follow-up and to the distant sites in 3 patients. At the time of the last contact on November 30, 2020, 4 patients were alive and disease-free, 7 had died of disease, and 6 had died of other causes. The case fatality rate was 41.2%. Five-year disease-free and disease-specific survivals were 51.8% and 59.7%, respectively. The 5-year disease-specific survival was 75% for early stage MCC (stage I and II) and 35.7% for stage III MCC. Early diagnosis and intervention are crucial for disease control and improving survival.


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.


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