Left-sided laterality of Merkel cell carcinoma in a German population: more than just sun exposure

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).

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Merkel Cell Carcinoma: New Trends

Cancers ◽

10.3390/cancers13071614 ◽

2021 ◽

Vol 13 (7) ◽

pp. 1614

Author(s):

Ellen M. Zwijnenburg ◽

Satish F.K. Lubeek ◽

Johanna E.M. Werner ◽

Avital L. Amir ◽

Willem L.J. Weijs ◽

...

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Tumor Biology ◽

Sun Exposure ◽

The Elderly ◽

Treatment Modalities ◽

Merkel Cell ◽

Immunohistochemical Markers ◽

Diagnostic Work ◽

Work Up

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin mainly seen in the elderly. Its incidence is rising due to ageing of the population, increased sun exposure, and the use of immunosuppressive medication. Additionally, with the availability of specific immunohistochemical markers, MCC is easier to recognize. Typically, these tumors are rapidly progressive and behave aggressively, emphasizing the need for early detection and prompt diagnostic work-up and start of treatment. In this review, the tumor biology and immunology, current diagnostic and treatment modalities, as well as new and combined therapies for MCC, are discussed. MCC is a very immunogenic tumor which offers good prospects for immunotherapy. Given its rarity, the aggressiveness, and the frail patient population it concerns, MCC should be managed in close collaboration with an experienced multidisciplinary team.

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Merkel Cell Carcinoma Presenting as an Indurated Plaque

SKIN The Journal of Cutaneous Medicine ◽

10.25251/skin.4.6.10 ◽

2020 ◽

Vol 4 (6) ◽

pp. 559-562

Author(s):

Margaret Coleman Maxi ◽

Joel Byrne ◽

Ardenne Martin ◽

Pamela Martin

Keyword(s):

Cell Carcinoma ◽

Chest Wall ◽

Merkel Cell Carcinoma ◽

Unusual Case ◽

Case Reports ◽

Sun Exposure ◽

Outpatient Setting ◽

Merkel Cell ◽

Threefold Increase ◽

History Of

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.

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LSD1 inhibitors induce neuronal differentiation of Merkel cell carcinoma by disrupting the LSD1-CoREST complex and activating TGFβ signaling

10.1101/2020.04.14.041657 ◽

2020 ◽

Author(s):

Lukas Leiendecker ◽

Pauline S. Jung ◽

Tobias Neumann ◽

Thomas Wiesner ◽

Anna C. Obenauf

Keyword(s):

Cell Carcinoma ◽

Cell Fate ◽

Merkel Cell Carcinoma ◽

Gene Expression Signature ◽

Sun Exposure ◽

Merkel Cell ◽

Entry Points ◽

Epigenetic Regulators

AbstractMerkel cell carcinoma (MCC) is a highly aggressive, neuroendocrine skin cancer that is either associated with the clonal integration of the Merkel cell polyomavirus or with chronic sun exposure1,2. Immunotherapy is initially effective in many patients with metastatic MCC, but the response is rarely durable3,4. MCC lacks actionable mutations that could be utilized for targeted therapies, but epigenetic regulators, which govern cell fate, provide unexplored therapeutic entry points. Here, we performed a pharmacological screen in MCC cells, targeting epigenetic regulators. We discovered that the lysine-specific histone demethylase 1A (LSD1/KDM1A) is required for MCC growth in vitro and in vivo. HMG20B (BRAF35), a poorly characterized subunit of the LSD1-CoREST complex, is also essential for MCC proliferation. LSD1 inhibition in MCC disrupts the LSD1-CoREST complex, directly induces the expression of key regulators of the neuronal lineage and of members of the TGFβ pathway, and activates a gene expression signature corresponding to normal Merkel cells. Our results provide a rationale for evaluating LSD1 inhibitors, which are currently being tested in patients with leukemia and solid tumors, in MCC.

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Merkel Cell Carcinoma of the Head in a Young African Albino Woman with HIV/HTLV-1 Coinfection Associated with Multiple Squamous Cell Carcinomas

Case Reports in Dermatology ◽

10.1159/000499898 ◽

2019 ◽

Vol 11 (1) ◽

pp. 113-122

Author(s):

Daniela Tenea ◽

Jurgen Dinkel ◽

Jurgen C. Becker ◽

Elisabeth van der Walt

Keyword(s):

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Squamous Cell ◽

Sun Exposure ◽

Squamous Cell Carcinomas ◽

Sub Saharan Africa ◽

Merkel Cell ◽

Skin Cancers ◽

Sub Saharan ◽

B Lymphoid

Merkel cell carcinoma (MCC) is a rare cutaneous neoplasm of presumed neuroendocrine origin, with aggressive behavior and poor prognosis, that tends to have an increased incidence among elderly Caucasians and immunosuppressed individuals. MCC is either associated with a clonal integration of the Merkel cell polyoma virus into the host genome or with genomic alterations caused by chronic UV exposure. Tumors of either carcinogenesis show epithelial, neuroendocrine, and B-lymphoid lineage markers. HIV-infected African albinos have a higher risk of developing skin cancers, including MCC, in comparison with the general population. We report a case of MCC of the head in a young albino woman with a HIV/HTLV-1 coinfection. The patient also suffered from multiple squamous cell carcinomas of the scalp, face, lip, and ears, suggesting an UV carcinogenesis of MCC. The purpose of this case report is to emphasize the relationship between immunosuppression (HIV/HTLV-1 coinfection, chronic sun exposure, ocular-cutaneous albinism, pregnancy) and MCC. It highlights the importance of early diagnosis, dermatological screening with a risk-stratified surveillance, particularly in immunosuppressed albino patients in sub-Saharan Africa, and multidisciplinary management of this biologically unique cutaneous cancer.

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Merkel Cell Carcinoma of the Hand

Hand ◽

10.1177/1558944715616098 ◽

2016 ◽

Vol 11 (4) ◽

pp. NP24-NP29 ◽

Cited By ~ 3

Author(s):

Donevan R. Westerveld ◽

David J. Hall ◽

Winston T. Richards

Keyword(s):

Local Recurrence ◽

Cell Carcinoma ◽

Merkel Cell Carcinoma ◽

Multidisciplinary Treatment ◽

Proximal Phalanx ◽

Sun Exposure ◽

Distant Metastases ◽

Adjuvant Radiation ◽

Merkel Cell ◽

High Incidence

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.

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