Oncologic outcomes of patients with lymph node–positive Merkel cell carcinoma with unknown primary: A retrospective cohort study

Hui, Jane Yuet Ching; Prathibha, Saranya; White, McKenzie S.; Praska, Corinne; LaRocca, Christopher J.; Ankeny, Jacob S.; Domingo-Musibay, Evidio; Fiessinger, Lori; Jensen, Eric H.; Tuttle, Todd M; Marmor, Schelomo

doi:10.1016/j.jaad.2022.06.015

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…While MCC most commonly presents as a skin lesion, cases of metastatic MCC without a primary skin lesion have also been sporadically reported (4). Furthermore, it is exceptionally rare for MCC to manifest solely in superficial lymph nodes without accompanying skin lesions or distant metastases (5).…”

Section: Introductionmentioning

confidence: 99%

Primary Nodal Merkel Cell Carcinoma: Rare Presentation

Tekin

Özen

Gürbüz

2023

Osmangazi̇ Journal of Medicine

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Merkel cell carcinoma (MCC) is a very rare high-grade neuroendocrine carcinoma that is frequently located in the skin. Although most cases are associated with Merkel cell polyomavirus, there are also cases in which the virus cannot be detected. The purpose of presenting the case of MCC with primary nodal involvement is due to its rare presentation. A 59-year-old male patient presented to the hospital with painless mass in the neck. Imaging analyses revealed necrotic lymphadenopathies on the neck, measuring up to 4.5 cm in size. After an explorative surgery with preliminary diagnoses of lymphoma and squamous cell carcinoma , samples taken from the excised mass showed high-grade neuroendocrine carcinoma morphology. In addition to neuroendocrine markers, CK7 and CK20 positivity were observed in the immunohistochemical tests performed. As a result, the case was evaluated in favor of MCC with negativity of markers performed to exclude other malignancies. Subsequently, a diagnosis of primary nodal MCC was made due to the absence of a different focus other than lymph node on PET imaging analysis. The differential diagnosis should be kept broad due to the histopathological morphology of small round cell malignant tumors. In addition in high-grade neuroendocrine carcinomas, the possibility of MCC should be ruled out especially if CK20 positivity is present. Although is has been reported that primary nodal MCC cases have a better clinical course compared to nodal metastatic cases with a known primary, there are limited case series studies on this topic. A multisystem approach is recommended for proper clinical management.

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Section: Introductionmentioning

confidence: 99%

Primary Nodal Merkel Cell Carcinoma: Rare Presentation

Tekin

Özen

Gürbüz

2023

Osmangazi̇ Journal of Medicine

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Nodal Merkel Cell Carcinoma with Unknown Primary Site and No Distant Metastasis: A Single-Center Series

Fazio

Maisonneuve

Spada

et al. 2022

Cancers

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Merkel cell carcinoma (MCC) is a very rare and aggressive neuroendocrine carcinoma originating from Merkel cells, typically with a skin nodule; however, it exceptionally presents with only a basin lymph node localization, with neither a cutaneous primary site nor distant metastases. From 1996 to 2020, among patients with histologically confirmed MCC managed at a neuroendocrine neoplasm-referral center, we selected those with an exclusive nodal basin, no distant metastasis, and an unknown primary site defined by cross-sectional and physical examination. A total of 55 out of 310 patients fulfilled the selection criteria. The median age was 64 years and the majority were males. Inguinal lymph-nodes were the most common anatomic site. With a median follow-up of 4.3 years, the 5-year relapse-free survival (RFS) rate was 56.6 (95% CI 42.0–68.8%) and the 5-year cancer specific survival (CSS) rate was 68.5 (95% CI 52.8–79.9%) for the whole population. The 36 patients (65.5%) undergoing lymphadenectomy (LND) + radiotherapy (RT) ± chemotherapy had a 5-year RFS rate of 87.2% (95% CI 65.5–95.7%) and a 5-year CSS rate of 90.5% (95% CI 67.0–97.5), which were better than those receiving LND alone. In a multivariable analysis, the survival benefit for LND + RT remained significant. Results from one of the largest single-center series of nMCC-UP suggest that a curative approach including RT can be effective, similar to what is observed for stage IIIB MCC. Multicentric studies with homogenous populations should be carried out in this controversial clinical entity, to minimize the risk of biases and provide robust data.

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Oncologic outcomes of patients with lymph node–positive Merkel cell carcinoma with unknown primary: A retrospective cohort study

Cited by 2 publications

References 5 publications

Primary Nodal Merkel Cell Carcinoma: Rare Presentation

Primary Nodal Merkel Cell Carcinoma: Rare Presentation

Nodal Merkel Cell Carcinoma with Unknown Primary Site and No Distant Metastasis: A Single-Center Series

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