scholarly journals Merkel Cell Carcinoma Presenting as an Indurated Plaque

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.  

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).


2019 ◽  
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  

Abstract Background Merkel cell carcinoma (MCC) is a rare primary neuroendocrine cutaneous tumor, rarely metastatizing to the brain. Chronic lymphoid leukemia (CLL) is a disease predisposing to MCC. According to previous reports, headache and focal neurological deficits suggest disease progression to the brain. We present a patient with MCC whose seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. Case presentation The 62-year-old female patient had a history of CLL. A lesion with the appearance of an atheroma was removed from the right upper arm. Histology confirmed the diagnosis of MCC. She was admitted to the neurology department with her first GM seizure. The cranial MRI/MRA showed bone metastases in the right parietal and left frontal areas, compressing the brain. Flow cytometry of CSF did not reveal metastasis of MCC or CLL. No surgery was performed, chemotherapy was continued. Conclusions The case history of the patient was unique even among the rare cases of MCC with neurological involvement. The seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. In addition to patient history, clinical presentation and radiological findings enabled a suspected diagnosis preceding confirmation by laboratory methods.


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.


2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
András Folyovich ◽  
Angéla Majoros ◽  
Tamás Jarecsny ◽  
Gitta Pánczél ◽  
Zsuzsanna Pápai ◽  
...  

Background. Merkel cell carcinoma (MCC) is a rare primary neuroendocrine cutaneous tumor, rarely metastasizing to the brain. Chronic lymphoid leukemia (CLL) is a disease predisposing to MCC. According to previous reports, headache and focal neurological deficits suggest disease progression to the brain. We present a patient with MCC whose seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. Case Presentation. A 62-year-old female patient had a history of CLL. A lesion with the appearance of an atheroma was removed from the right upper arm. Histology confirmed the diagnosis of MCC. She was admitted to the neurology department with her first GM seizure. The cranial MRI/MRA showed bone metastases in the right parietal and both frontal areas, compressing the brain. Flow cytometry of CSF did not reveal metastasis of MCC. Conclusions. The case history of the patient was unique even among the rare cases of MCC with neurological involvement. The seizure was not elicited by a cerebral metastasis, but by bone metastases compressing the brain. In addition to patient history, clinical presentation and radiological findings enabled a suspected diagnosis of skull metastasis of MCC compressing the brain, causing symptomatic epileptic seizures.


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.


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.


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.


2005 ◽  
Vol 23 (10) ◽  
pp. 2300-2309 ◽  
Author(s):  
Peter J. Allen ◽  
Wilbur B. Bowne ◽  
David P. Jaques ◽  
Murray F. Brennan ◽  
Klaus Busam ◽  
...  

PurposeMerkel cell carcinoma (MCC) is an uncommon cutaneous malignancy. Most reports consist of single-institution experiences of fewer than 30 patients. The natural history of MCC is poorly defined.Patients and MethodsA review was performed of Memorial Sloan-Kettering Cancer Center's MCC database, identifying 251 patients who had been treated between 1970 and 2002. Patient, tumor, and treatment-related factors were analyzed for their association with recurrence and survival.ResultsThe average follow-up for all patients was 40 months and 46 months for patients alive at last follow-up. The 5-year disease-specific survival rate was 64%. Disease stage was the only independent predictor of survival (stage I, 81%; stage II, 67%; stage III, 52%; stage IV, 11%; P = .001). Pathologic staging of the draining nodal basin was performed in 71 (40%) of 177 patients who presented with clinically negative nodes, and 16 of these patients (23%) were found to have node-positive disease. Pathologic nodal staging was associated with improved stage-specific survival probabilities (clinical node-negative, 75% v pathologic node-negative disease, 97%; P = .009) and decreased nodal recurrence (44% v 11%, P < .001). The median time to recurrence was 9 months, and 102 patients (43%) recurred. Local recurrence developed in 8% of patients after margin-negative excision.ConclusionThese data demonstrate that the natural history of MCC is variable and dependent on the stage of disease at presentation. Pathologic nodal staging identifies a group of patients with excellent long-term survival. After margin-negative excision and pathologic nodal staging, local and nodal recurrence rates are low.


2005 ◽  
Vol 114 (5) ◽  
pp. 376-380 ◽  
Author(s):  
Luis Junquera ◽  
Aintza Torre ◽  
Luis García-Consuegra ◽  
Juan C. Vicente ◽  
Manuel F. Fresno

Merkel cell carcinoma (MCC) is a very aggressive primary cutaneous neoplasm most often occurring on the head and neck of the elderly. Spontaneous regression of MCC was first described in 1986. A 79-year-old woman with MCC on the right cheek underwent spontaneous regression of the malignancy, documented by photographic follow-up, computed tomography, and histologic studies. A review of the literature is presented. Complete clinical and histologic regression of MCC was observed in the present case. Although the literature documents 11 similar cases, only 6 can be regarded as complete spontaneous regressions following exclusive performance of a biopsy (primary complete spontaneous regression). Primary complete spontaneous regression of MCC is infrequent, and most case reports describe this phenomenon in women with MCC on the cheek. The reasons underlying regression are unknown.


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