A Case of Merkel Cell Carcinoma of the External Auditory Canal Skin with Annulus and Tympanic Membrane

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.

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RE: Adjuvant Radiation Therapy and Chemotherapy in Merkel Cell Carcinoma: Survival Analyses of 6908 Cases From the National Cancer Data Base

JNCI Journal of the National Cancer Institute ◽

10.1093/jnci/djx052 ◽

2017 ◽

Vol 109 (10) ◽

Cited By ~ 2

Author(s):

John A. Vargo ◽

Erica R. Ghareeb ◽

Goundappa K. Balasubramani ◽

Sushil Beriwal

Keyword(s):

Radiation Therapy ◽

Cell Carcinoma ◽

Data Base ◽

Merkel Cell Carcinoma ◽

National Cancer Data Base ◽

Adjuvant Radiation ◽

Merkel Cell ◽

Survival Analyses ◽

Adjuvant Radiation Therapy ◽

Cancer Data

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Epileptic Seizure Provoked by Bone Metastasis of Chronic Lymphoid Leukemia and Merkel Cell Carcinoma – Case Report

10.21203/rs.2.358/v1 ◽

2019 ◽

Author(s):

András Folyovich ◽

Angéla Majoros ◽

Tamás Jarecsny ◽

Gitta Pánczél ◽

Zsuzsanna Pápai ◽

...

Keyword(s):

Bone Metastases ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Cerebral Metastasis ◽

Merkel Cell ◽

Lymphoid Leukemia ◽

Chronic Lymphoid Leukemia ◽

History Of ◽

The Right ◽

The Brain

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.

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Merkel Cell Carcinoma: Recent Progress and Current Priorities on Etiology, Pathogenesis, and Clinical Management

Journal of Clinical Oncology ◽

10.1200/jco.2009.22.6605 ◽

2009 ◽

Vol 27 (24) ◽

pp. 4021-4026 ◽

Cited By ~ 85

Author(s):

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Clinical Management ◽

Expression Profiles ◽

T Antigen ◽

Neuroendocrine Cells ◽

Human Polyomavirus ◽

Adjuvant Radiation ◽

Cytokeratin 20 ◽

Merkel Cell

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.

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Faculty Opinions recommendation of Narrow excision margins are appropriate for Merkel cell carcinoma when combined with adjuvant radiation: Analysis of 188 cases of localized disease and proposed management algorithm.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.738394298.793586199 ◽

2021 ◽

Author(s):

Desiree Ratner

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Adjuvant Radiation ◽

Merkel Cell ◽

Management Algorithm ◽

Localized Disease ◽

Excision Margins

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Angiosarcoma in Collision With a Merkel Cell Carcinoma: Case Report and Literature Review

International Journal of Surgical Pathology ◽

10.1177/1066896919880260 ◽

2019 ◽

Vol 28 (3) ◽

pp. 325-329

Author(s):

Sean Hacking ◽

Hector Chavaria ◽

Leonard Khan ◽

Kasturi Das

Keyword(s):

Radiation Therapy ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Collision Tumor ◽

Molecular Profile ◽

Rare Entity ◽

Merkel Cell ◽

Elderly Males ◽

History Of ◽

Rare Malignancy

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.

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Epileptic Seizure Provoked by Bone Metastasis of Chronic Lymphoid Leukemia and Merkel Cell Carcinoma

Case Reports in Medicine ◽

10.1155/2020/4318638 ◽

2020 ◽

Vol 2020 ◽

pp. 1-4

Author(s):

András Folyovich ◽

Angéla Majoros ◽

Tamás Jarecsny ◽

Gitta Pánczél ◽

Zsuzsanna Pápai ◽

...

Keyword(s):

Bone Metastases ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Cerebral Metastasis ◽

Merkel Cell ◽

Lymphoid Leukemia ◽

Chronic Lymphoid Leukemia ◽

History Of ◽

The Right ◽

The Brain

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.

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Merkel Cell Carcinoma: High Recurrence Rate Despite Aggressive Treatment

Journal of Surgical Research ◽

10.1016/j.jss.2011.11.135 ◽

2012 ◽

Vol 172 (2) ◽

pp. 194

Author(s):

M.C. Soult ◽

E.C. Feliberti ◽

M.L. Silverberg ◽

R.R. Perry

Keyword(s):

Cell Carcinoma ◽

Recurrence Rate ◽

Merkel Cell Carcinoma ◽

High Recurrence Rate ◽

Merkel Cell ◽

Aggressive Treatment

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Unknown primary Merkel cell carcinoma with cutaneous spread

BMJ Case Reports ◽

10.1136/bcr-2018-224834 ◽

2019 ◽

Vol 12 (2) ◽

pp. e224834

Author(s):

João Vasco Barreira ◽

Margarida Moura Valejo Coelho ◽

Catarina Ribeiro ◽

Mónica Semedo

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Primary Tumour ◽

Left Knee ◽

Unknown Primary ◽

Multidisciplinary Teams ◽

Histopathological Analysis ◽

Merkel Cell ◽

Dermatological Examination ◽

History Of

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.

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