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

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.

scholarly journals Squamous Cell Carcinoma in African Children with Xeroderma Pigmentosum: Three Case Reports

2016 ◽  
Vol 8 (3) ◽  
pp. 311-318 ◽  
Author(s):  
Mamadou Kaloga ◽  
Pauline Dioussé ◽  
Boubacar Ahy Diatta ◽  
Mariama Bammo ◽  
Sarah Kourouma ◽  
...  
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Squamous Cell ◽  
Xeroderma Pigmentosum ◽  
Case Reports ◽  
Sub Saharan Africa ◽  
Skin Cancers ◽  
Black Skin ◽  
The Mean ◽  
Sub Saharan

Introduction: Xeroderma pigmentosum is a rare autosomal recessive genetic disease. This disease predisposes patients to early-onset skin cancers, particularly squamous cell carcinoma. Here, we report 3 pediatric cases, including 2 deaths. Observation: The subjects included 2 boys and 1 girl with skin type VI. All subjects were from consanguineous marriages, and the average age was 7.6 years. The patients all had ulcerative budding tumor lesions in the cephalic region, and the mean disease duration was 18 months. In all 3 cases, the diagnosis of xeroderma pigmentosum was made before the poikilodermal appearance of sun-exposed areas and photophobia. Neurological-type mental retardation was noted in 1 case. Histology confirmed squamous cell carcinoma in all 3 cases. The evolutions were marked by the death of 2 children (cases 1 and 3). In one case, the outcome was favorable following cancer excision and subsequent chemotherapy with adjuvant radiotherapy. Conclusion: Squamous cell carcinoma is a serious complication related to xeroderma pigmentosum in Sub-Saharan Africa. Prevention is based on the early diagnosis of xeroderma pigmentosum, black skin photoprotection, screening and early treatment of lesions, and genetic counseling.

scholarly journals Regionally Metastatic Merkel Cell Carcinoma Associated with Paraneoplastic Anti-N-methyl-D-aspartate Receptor Encephalitis

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Sophia Z. Shalhout ◽  
Kevin S. Emerick ◽  
Peter M. Sadow ◽  
Jenny J. Linnoila ◽  
David M. Miller
Keyword(s):  
Cell Carcinoma ◽  
Merkel Cell Carcinoma ◽  
Psychiatric Symptoms ◽  
Checkpoint Inhibitors ◽  
Case Reports ◽  
Intensity Modulated Radiation Therapy ◽  
Sun Exposure ◽  
Merkel Cell ◽  
Paraneoplastic Encephalitis ◽  
Aspartate Receptor

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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