Concurrent Metastatic Merkel Cell Carcinoma and Cutaneous Squamous Cell Carcinoma in the Same Lymph Node

Suaiti, Lubna; Onajin, Oluwakemi; Sangüeza, Omar P.

doi:10.1097/dad.0000000000001318

Cited by 5 publications

(11 citation statements)

References 16 publications

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“…Suaiti et al described their case as a collision tumor of MCC and SCC, where both components happened to metastasize to the same lymph node. As in some other previous studies, they hypothesize that the origin of combined MCC could be related to the hair follicle 14 . On the other hand, Martin et al considered their combined MCC and SCC cases as tumors derived from a common precursor cell with divergent differentiation, their pathogenesis being unrelated to MCPyV 3 .…”

Section: Discussionmentioning

confidence: 83%

“…Although the exact pathogenesis of these combined tumors is still unknown, it appears that they represent a distinct disease process from pure MCC, as they are mostly MCPyV negative, and appear to show a more aggressive behavior 3–5,8,10,11 . Moreover, two recent case reports have demonstrated the presence of both neuroendocrine and squamous components in nodal metastases of combined MCC and SCC tumors, probably pointing to a common precursor cell 12–14 . We present two additional cases of combined MCC and SCC with nodal metastases in which both cellular lineages were present, both in the primary tumor and in the metastasis.…”

Section: Introductionmentioning

confidence: 78%

“…As in some other previous studies, they hypothesize that the origin of combined MCC could be related to the hair follicle. 14 On the other hand, Martin et al considered their combined MCC and SCC cases as tumors derived from a common precursor cell with divergent differentiation, their pathogenesis being unrelated to MCPyV. 3 Finally, Navarrete et al do not come to any conclusion regarding the origin of the neuroendocrine and squamous elements of these tumors, but they do highlight that it is unrelated to MCPyV infection.…”

Section: Casementioning

confidence: 99%

“…[3][4][5]8,10,11 Moreover, two recent case reports have demonstrated the presence of both neuroendocrine and squamous components in nodal metastases of combined MCC and SCC tumors, probably pointing to a common precursor cell. [12][13][14] We present two additional cases of combined MCC and SCC with nodal metastases in which both cellular lineages were present, both in the primary tumor and in the metastasis. Interestingly, one of them was MCPyV negative, while the other case was found to be MCPyV positive.…”

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confidence: 96%

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Combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases: Report of two cases

et al. 2022

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Merkel cell carcinoma (MCC) is an infrequent, aggressive cutaneous neoplasm, that typically affects the photodamaged skin of elderly individuals, and immunosuppressed patients. Because a subset of MCC is closely related to UV radiation, MCC can develop concurrently with other tumors, most commonly, as a combined tumor with squamous cell carcinoma (SCC). These combined tumors appear to represent a distinct disease process from pure MCC, as they are mostly Merkel cell polyomavirus (MCPyV) negative, and show a more aggressive behavior. We present two additional cases of combined MCC and SCC with nodal metastases, one of which was MCPyV positive. Two different subtypes of MCC have been proposed based on their origin: a true neuroendocrine carcinoma, that is MCPyV positive and has a dermal origin, and a UV-related SCC with neuroendocrine differentiation. This theory could explain why MCC can develop concurrently with SCC, and why these combined cases are generally MCPyV negative. However, it fails to explain the minority of combined MCC and SCC tumors that are MCPyV positive. Because both our patients had a history of chronic UV exposure, we hypothesize that UV radiation probably played a major role in the pathogenesis of these tumors, while MCPyV integration probably acted as an additional trigger.combined Merkel cell carcinoma, combined Merkel cell carcinoma and squamous cell carcinoma, Merkel cell carcinoma, Merkel cell polyomavirus, nodal metastasis, squamous cell carcinoma | INTRODUCTIONMerkel cell carcinoma (MCC), also known as primary cutaneous neuroendocrine tumor, is an infrequent, aggressive cutaneous neoplasm, that typically affects the photodamaged skin of elderly individuals, and patients with a history of immunosuppression. [1][2][3][4][5][6] Although the histogenesis of MCC has been the object of considerable debate since its original description, and the cell of origin is still unknown, two distinct subtypes of MCC have now been proposed based on their pathogenesis: one that is related to the Merkel cell polyomavirus (MCPyV), that represents the majority of all cases (with variable percentages, estimated at 80% based on some studies), hypothesized to be of possible dermal (fibroblastic) origin, and another one unrelated to MCPyV infection, that appears to be caused by UV radiation, considered to be of probable epidermal origin (keratinocytic). 1,2,[7][8][9] Because a significant subset of MCC is closely related to UV radiation, it is not unusual for this tumor to be found in close association (as a collision tumor) to other UV-related neoplasms, and this has been reported in the literature with variable frequency. On the other hand, combined MCC tumors (MCC that differentiating into other neoplasms, sharing a

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Section: Discussionmentioning

confidence: 83%

Section: Introductionmentioning

confidence: 78%

Section: Casementioning

confidence: 99%

mentioning

confidence: 96%

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Combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases: Report of two cases

et al. 2022

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“…[ 48 ] Suaiti et al presented a case of combined MCC in the left ear, in which both tumor components metastasized to the same lymph node of the parotid during immunosuppression. [ 49 ] This combined MCC is considered a fortuitous collision between the MCC and SCC. A triple-collision tumor with MCC, SCC in situ, and basal cell carcinoma with an unusual CK20 negative and TTF-1 positive immunophenotype has also been reported.…”

Section: Originmentioning

confidence: 99%

A review on the oncogenesis of Merkel cell carcinoma: Several subsets arise from different stages of differentiation of stem cell

Zhu

Yin

et al. 2023

Medicine

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Merkel cell carcinoma (MCC), a rare primary cutaneous neuroendocrine neoplasm, is extremely aggressive and has a higher mortality rate than melanoma. Based on Merkel cell polyomavirus (MCPyV) status and morphology, MCCs are often divided into several distinct subsets: pure MCPyV-positive, pure MCPyV-negative, and combined MCC. MCPyV-positive MCC develops by the clonal integration of viral DNA, whereas MCPyV-negative MCC is induced by frequent ultraviolet (UV)-mediated mutations, that are characterized by a high mutational burden, UV signature mutations, and many mutations in TP53 and retinoblastoma suppressor gene ( RB1 ). Combined MCC consists of an intimate mix of MCC and other cutaneous tumor populations, and is usually MCPyV-negative, with rare exceptions. Based on the existing subsets of MCC, it is speculated that there are at least 4 stages in the natural history of stem cell differentiation: primitive pluripotent stem cells, divergent differentiated stem cells, unidirectional stem cells, and Merkel cells (or epidermal/adnexal cells). In the first stage, MCPyV may integrate into the genome of primitive pluripotent stem cells, driving oncogenesis in pure MCPyV-positive MCC. If MCPyV integration does not occur, the stem cells enter the second stage and acquire the ability to undergo multidirectional neuroendocrine and epidermal (or adnexal) differentiation. At this stage, accumulated UV-mediated mutations may drive the development of combined MCC. In the third stage, the stem cells differentiate into unidirectional neuroendocrine stem cells, UV-mediated mutations can induce carcinogenesis in pure MCPyV-negative MCC. Therefore, it has been speculated that several subsets of MCCs arise from different stages of differentiation of common stem cells.

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Primary cutaneous phalangeal neuroendocrine/squamous cell carcinoma with mixed axillary metastasis

et al. 2022

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Cutaneous neuroendocrine tumours are rare and aggressive tumours associated with advanced age and immunosuppression. They are typically characterised by a high rate of local recurrence and nodal disease. The presence of a mixed squamous cell component is rare. These tumours are uncommonly found on the hand. We present a case and histological images of a 78-year-old woman with a primary CK20 negative TTF-1 positive cutaneous neuroendocrine tumour with squamous dedifferentiation arising from the fifth digit with axillary metastasis showing a mixed phenotype. Initial biopsy of the lesion was positive for chromogranin, synaptophysin and TTF-1, but negative for CK20, Melan-A and S100. After CT of the thorax abdomen and pelvis and octreotide single positron emission CT demonstrated a 15 mm axillary metastasis and no evidence of distal disease, our patient underwent an amputation of the affected digit and an axillary lymph node dissection. She is currently awaiting adjuvant chemoradiotherapy. Only two cases are reported in the literature to have mixed squamous/neuroendocrine features. We present the first case which is CK20 negative and TTF-1 positive.

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Concurrent Metastatic Merkel Cell Carcinoma and Cutaneous Squamous Cell Carcinoma in the Same Lymph Node

Cited by 5 publications

References 16 publications

Combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases: Report of two cases

Combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases: Report of two cases

A review on the oncogenesis of Merkel cell carcinoma: Several subsets arise from different stages of differentiation of stem cell

Primary cutaneous phalangeal neuroendocrine/squamous cell carcinoma with mixed axillary metastasis

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