RESUMEN
ABSTRACT: Merkel cell carcinoma (MCC) is an uncommon highly aggressive cutaneous neuroendocrine neoplasm with high mortality. Rarer still is nasopharyngeal metastasis of MCC. Herein, we report the 68Ga-DOTATATE PET/CT findings of MCC with metastasis to the nasopharynx in a 53-year-old man who underwent surgery for MCC in his thigh 2 years ago.
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Carcinoma de Células de Merkel , Neoplasias Nasofaríngeas , Compuestos Organometálicos , Tomografía Computarizada por Tomografía de Emisión de Positrones , Humanos , Carcinoma de Células de Merkel/diagnóstico por imagen , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/secundario , Masculino , Persona de Mediana Edad , Neoplasias Nasofaríngeas/diagnóstico por imagen , Neoplasias Nasofaríngeas/patologíaRESUMEN
Approximately 5-15% of all dermatologic malignancies manifest in the upper and lower eyelids. The primary types include basal cell carcinoma, squamous cell carcinoma, and sebaceous cell carcinoma, with Merkel cell carcinoma and melanoma following closely behind. Basal cell carcinoma predominantly affects the lower eyelid, yet various other carcinomas, melanomas, metastases, and neoplasms of diverse origins can arise on both upper and lower eyelids. Risk factors such as advanced age, smoking, and notably, exposure to UV light significantly contribute to the development of these eyelid lesions. Despite the increasing incidence, research on dermatologic eyelid malignancies remains limited. However, such study is imperative given that many systemic oncologic malignancies initially present as metastatic eyelid lesions. This paper provides an in-depth exploration of eyelid anatomy, clinical presentation, diagnosis, and treatment management.Key Points: Eyelid metastases represent less than one percent of all eyelid cancers, yet they often serve as the initial indication of an underlying systemic malignancy. Early detection and treatment is crucial in improving prognosis and quality of life for patients. Treatment options encompass a range of modalities, with Mohs surgery as the gold standard for the removal of ocular tumors. Additional treatment options include local excision as well as non-surgical interventions such as radiotherapy, cryotherapy, immunotherapy, and topical medications.
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Neoplasias de los Párpados , Humanos , Neoplasias de los Párpados/terapia , Neoplasias de los Párpados/diagnóstico , Neoplasias de los Párpados/epidemiología , Neoplasias de los Párpados/patología , Párpados/patología , Cirugía de Mohs , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/epidemiología , Melanoma/terapia , Melanoma/diagnóstico , Melanoma/patología , Melanoma/epidemiología , Carcinoma de Células de Merkel/terapia , Carcinoma de Células de Merkel/diagnóstico , Carcinoma de Células de Merkel/epidemiología , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/patología , Factores de Riesgo , Carcinoma Basocelular/terapia , Carcinoma Basocelular/diagnóstico , Carcinoma Basocelular/epidemiología , Carcinoma Basocelular/patología , Carcinoma Basocelular/secundario , Calidad de Vida , Carcinoma de Células Escamosas/terapia , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/secundario , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/epidemiología , Neoplasias de las Glándulas Sebáceas/terapia , Neoplasias de las Glándulas Sebáceas/diagnóstico , Neoplasias de las Glándulas Sebáceas/patologíaRESUMEN
Merkel cell carcinoma (MCC) is an uncommon and aggressive skin cancer of neuroendocrine origin. The tumor usually presents with a locoregional spread and most frequently metastasizes to the skin, liver, bone, lung, and brain. Despite the orbit being a relatively common site of metastases, it has rarely been reported in patients with MCC. The authors present a case of biopsy-proven orbital metastatic MCC in an 86-year-old male who presented with a rapidly enlarging right caruncle/subconjunctival mass with orbital extension and a history of forearm MCC excision 3 years prior. There are only 3 reported cases of distant metastatic MCC to the orbit, all presenting as a mass originating from extraocular muscles; and no cases of caruncle involvement.
Asunto(s)
Carcinoma de Células de Merkel , Neoplasias Orbitales , Neoplasias Cutáneas , Humanos , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/diagnóstico , Masculino , Anciano de 80 o más Años , Neoplasias Orbitales/secundario , Neoplasias Orbitales/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/secundario , Neoplasias Cutáneas/diagnóstico , Neoplasias de la Conjuntiva/secundario , Neoplasias de la Conjuntiva/diagnóstico , Neoplasias de la Conjuntiva/patología , Biopsia , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive and rare neuroendocrine tumor, accounting for less than 1% of skin cancers. Metastasis primarily manifests in the cervical lymph nodes but rarely affect the thyroid. METHODS: We report a case of primary head and neck cutaneous MCC with metastasis to the thyroid gland. A review of the literature of MCC with thyroid metastasis was conducted. RESULTS: We identified five cases of MCC with thyroid metastasis. Primary sites included the distal upper and lower extremities, axilla, buttock, and groin. Treatment courses varied including thyroidectomy, immunotherapy, and expectant palliative measures. Time from initial diagnosis to thyroid metastasis ranged from four months to four years. Tissue diagnosis was achieved in 5 of 6 cases. CONCLUSIONS: MCC with thyroid metastasis is rare and likely represents aggressive disease. Despite advances in treatment and surveillance, outcomes for MCC remain poor. Ongoing research may establish predictors for treatment response.
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Carcinoma de Células de Merkel , Neoplasias Cutáneas , Neoplasias de la Tiroides , Femenino , Humanos , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/terapia , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/terapia , Neoplasias de la Tiroides/patología , Neoplasias de la Tiroides/secundario , Neoplasias de la Tiroides/terapia , Tiroidectomía , Anciano de 80 o más AñosRESUMEN
Merkel cell carcinoma (MCC) is an unusual skin tumor that has a significant rate of distant and local metastases. It is known that primary MCC of the eyelid usually occurs at the upper eyelid. Here we report an unusual case of MCC metastasis to the eyelid. A 63-year-old male was diagnosed with MCC three years earlier after initially presenting with a mass in his right thigh. After histopathological diagnosis, the patient received medical therapy. During treatment, he developed multiple distant metastases and a firm, purple, vascularized lesion on the upper eyelid. We confirmed the lesion was an eyelid metastasis of MCC by histopathological examination and imaging methods. This case shows that extraocular MCC can metastasize to the eyelids, particularly the upper eyelid, where primary periocular MCC usually appears.
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Carcinoma de Células de Merkel , Neoplasias de los Párpados , Neoplasias Cutáneas , Humanos , Masculino , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/diagnóstico , Persona de Mediana Edad , Neoplasias de los Párpados/secundario , Neoplasias de los Párpados/diagnóstico , Neoplasias Cutáneas/secundario , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Biopsia , Párpados/patologíaRESUMEN
BACKGROUND: Merkel cell carcinoma (MCC) is associated with high rates of recurrence and distant metastatic progression. Current guidelines for surveillance imaging are not evidence based. Better characterization of the pattern of distant metastatic spread will better inform surveillance and facilitate earlier detection of metastases. OBJECTIVES: This retrospective study aimed to assess potential relationships between primary tumour site and site of initial distant metastasis, time to distant metastasis, overall survival (OS) and MCC-specific death (MSD). METHODS: Patients with local or regional (Stage I-III) disease who were treated with curative intent and progressed to Stage IV were included in this study (n = 151). Fisher's exact test was used to assess differences in patterns of initial distant metastases based on primary tumour site. Time to initial distant metastasis was calculated from date of MCC diagnosis. OS and MSD were calculated from date of initial distant metastasis to date of death from any or MCC-related causes, respectively. RESULTS: Of 151 patients included in analysis, 89 (58.9%) had a single initial distant metastatic site, and 62 (41.1%) had multiple sites. Patients with upper limb primary tumours were significantly less likely to develop distant lymph node or liver metastases (p = 0.02 and 0.04, respectively). Median time to distant metastasis was 11 months (IQR 6.7-17.9 months). Median OS was 15.3 months, and was shorter for patients with liver (7.0 months, p = 0.0004) or bone metastases (8.9 months, p < 0.0001). Using skin/soft tissue metastasis as a reference group, patients with multiple metastatic sites had significantly higher hazards of MSD (HR = 3.46 univariate, 3.77 multivariate analysis). Time to distant metastasis, OS and MSD did not differ by viral status. CONCLUSION: Sites of initial distant metastasis are related to primary tumour sites and survival outcomes. Because patients often have multiple initial metastases, full-body cross-sectional rather than region-specific imaging may facilitate earlier detection of metastatic disease.
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Carcinoma de Células de Merkel , Neoplasias Cutáneas , Humanos , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/terapia , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/mortalidad , Masculino , Femenino , Anciano , Estudios Retrospectivos , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/mortalidad , Anciano de 80 o más Años , Persona de Mediana Edad , Metástasis Linfática , Metástasis de la Neoplasia , Neoplasias Hepáticas/secundario , Estadificación de NeoplasiasRESUMEN
OBJECTIVE/BACKGROUND: Merkel cell carcinoma (MCC) but metastases to the pancreas are very rare. There are only a few cases of isolated metastases of MCC to the pancreas. Because of this rarity, it can be wrongly diagnosed as a neuroendocrine tumor of the pancreas(pNET), especially the poorly differentiated neuroendocrine carcinoma (PNEC) subtype, in which the treatment is vastly different than that of MCC with isolated metastases of the pancreas. METHODS: An electronic search of the PubMed and google scholar databases was performed to obtain the literature on MCC with pancreatic metastases, using the following search terms: Merkel cell carcinoma, pancreas, and metastases. Results are limited to the following available article types: case reports and case series. We identified 45 cases of MCC with pancreatic metastases from the PubMed and Google Scholar database search and examined their potential relevance. Only 22 cases with isolated pancreatic metastases were taken for review including one case that we encountered. RESULTS: The results from our review of cases of isolated pancreatic metastases of MCC were compared to the characteristics of the poorly differentiated pancreatic neuroendocrine tumor (PNEC). We found the following: (a) MCC with isolated pancreatic metastases occurred at an older age than PNEC and with male gender predominance (b) Most of the metastases occurred within 2 years of initial diagnosis of MCC (c) Resection of pancreatic mass was the first line treatment in case of resectable PNECs whereas resection of metastases was infrequently performed in MCC with pancreatic metastases.
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Carcinoma de Células de Merkel , Tumores Neuroendocrinos , Neoplasias Pancreáticas , Neoplasias Cutáneas , Masculino , Humanos , Carcinoma de Células de Merkel/diagnóstico , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/secundario , Neoplasias Cutáneas/diagnóstico , Tumores Neuroendocrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/patología , Páncreas/patologíaRESUMEN
A 60-year-old man presented with a painless, rapidly growing, haemorrhagic pink nodule on the posterior of his thigh that had developed 1 month previously. He had a diagnosis of IgA nephropathy and had received a renal allograft 7 years before. An excisional biopsy was performed and the diagnosis of Merkel cell carcinoma (MCC) was made. No distant metastases was detected. 10 months after first presentation, due to the development of acute pancytopenia and concomitant FDG PET/CT findings compatible with disease progression, bone marrow biopsy was performed which revealed metastasis of MCC. Dermatologists and oncologists should be aware that MCC could potentially involve the bone marrow in organ transplant recipients. In the follow-up period, a complete blood count should be carried out; FDG PET/CT can be obtained to follow up the metabolic status of the disease and bone marrow biopsy should be performed if necessary.
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Carcinoma de Células de Merkel , Trasplante de Riñón , Neoplasias Cutáneas , Masculino , Humanos , Persona de Mediana Edad , Carcinoma de Células de Merkel/diagnóstico , Carcinoma de Células de Merkel/secundario , Tomografía Computarizada por Tomografía de Emisión de Positrones , Fluorodesoxiglucosa F18 , Médula Ósea/patología , Neoplasias Cutáneas/diagnósticoRESUMEN
A 71-year-old male presented with Merkel cell carcinoma along with inguinal lymph node involvement (stage III). The patient was proposed for systemic treatment followed by inguinal lymphadenectomy and adjuvant radiotherapy. During the follow-up period, recurrences were documented (lymphatic and visceral) and were treated with salvage surgery and radiotherapy. On the fifth year of follow-up the patient was diagnosed with a metastasis in the right seminal vesicle and underwent stereotactic body radiation therapy. Two-years later, tumor recurrence in the right seminal vesicle was managed with salvage robotic assisted seminal vesiculectomy. Advanced stages of Merkel cell carcinoma have a poor outcome and salvage treatments should be tailored to each patient. A multidisciplinary approach was crucial in achieving successful outcomes. The patient is still recurrence free twenty-four months after surgery. To the best of our knowledge, this is the first publication reporting a seminal vesical Merkel cell metastasis.
Doente do sexo masculino de 71 anos, com carcinoma de células de Merkel com metastização ganglionar inguinal (estádio III), submetido a terapêutica sistémica seguida de linfadenectomia inguinal e radioterapia adjuvante. Durante o seguimento, todas as recorrências foram tratadas com radioterapia ou cirurgia de salvação. Cinco anos após o diagnóstico inicial, o doente foi diagnosticado com uma metástase na vesicula seminal direita, tendo sido tratado com radioterapia estereotáxica corporal. Dois anos mais tarde, por nova recorrência do tumor na vesicula seminal direita, foi submetido a uma vesiculectomia seminal robótica de salvação. Estádios avançados do carcinoma de células de Merkel apresentam um prognóstico reservado e os tratamentos de salvação devem ser adaptados a cada doente. Uma abordagem multidisciplinar revelou-se crucial para o sucesso do caso. O doente encontra-se no vigésimo quarto mês de pós-operatório, sem evidência de recidiva de doença. Até à data, este é o primeiro caso descrito de metástase na vesicula seminal de carcinoma de células de Merkel.
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Carcinoma de Células de Merkel , Procedimientos Quirúrgicos Robotizados , Neoplasias Cutáneas , Masculino , Humanos , Anciano , Carcinoma de Células de Merkel/cirugía , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/secundario , Terapia Recuperativa , Metástasis Linfática , Neoplasias Cutáneas/cirugía , Neoplasias Cutáneas/patología , Recurrencia Local de Neoplasia/patologíaRESUMEN
BACKGROUND: Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer that has a poor prognosis in patients with advanced disease. Avelumab [anti-programmed death-ligand 1 (PD-L1)] became the first approved treatment for patients with metastatic MCC (mMCC), based on efficacy and safety data observed in the JAVELIN Merkel 200 trial. We report long-term overall survival (OS) data after >5 years of follow-up from the cohort of patients with mMCC whose disease had progressed after one or more prior lines of chemotherapy. PATIENTS AND METHODS: In Part A of the single-arm, open-label, phase II JAVELIN Merkel 200 trial, patients with mMCC that had progressed following one or more prior lines of chemotherapy received avelumab 10 mg/kg by intravenous infusion every 2 weeks until confirmed disease progression, unacceptable toxicity, or withdrawal. In this analysis, long-term OS was analyzed. RESULTS: In total, 88 patients were treated with avelumab. At data cut-off (25 September 2020), median follow-up was 65.1 months (range 60.8-74.1 months). One patient (1.1%) remained on treatment, and an additional patient (1.1%) had reinitiated avelumab after previously discontinuing treatment. Median OS was 12.6 months [95% confidence interval (CI) 7.5-17.1 months], with a 5-year OS rate of 26% (95% CI 17% to 36%). In patients with PD-L1+ versus PD-L1- tumors, median OS was 12.9 months (95% CI 8.7-29.6 months) versus 7.3 months (95% CI 3.4-14.0 months), and the 5-year OS rate was 28% (95% CI 17% to 40%) versus 19% (95% CI 5% to 40%), respectively (HR 0.67; 95% CI 0.36-1.25). CONCLUSION: Avelumab monotherapy resulted in meaningful long-term OS in patients with mMCC whose disease had progressed following chemotherapy. These results further support the role of avelumab as a standard of care for patients with mMCC.
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Carcinoma de Células de Merkel , Neoplasias Cutáneas , Anticuerpos Monoclonales Humanizados/farmacología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Carcinoma de Células de Merkel/tratamiento farmacológico , Carcinoma de Células de Merkel/patología , Carcinoma de Células de Merkel/secundario , Estudios de Seguimiento , Humanos , Neoplasias Cutáneas/tratamiento farmacológicoRESUMEN
INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic has changed the lives of people around the world. Fortunately, sufficient vaccines are now available. Local reactions with ipsilateral lymphadenopathy are among the most common side effects. We investigated the impact of lymphadenopathy after COVID-19 vaccination on the value of ultrasound in tumour patients. PATIENTS AND METHODS: Patients with melanoma or Merkel cell carcinoma were included who underwent lymph node excision and received COVID-19 vaccination within 6 weeks before surgery. The consistency of the preoperative ultrasound findings with the histopathologic findings was investigated. RESULTS: Eight patients were included (two Merkel cell carcinoma and six melanoma patients) who underwent lymph node excision between 16th April 2021 and 19th May 2021 and had previously received COVID-19 vaccination. In three of the eight patients (one Merkel cell carcinoma and two melanoma patients), lymph node metastases were erroneously diagnosed preoperatively during tumour follow-up with physical examination, ultrasound, and or fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography (CT). In these three patients, the suspected lymph node metastases were located in the left axilla after COVID-19 vaccination in the left upper arm, which resulted in selective lymph node removal in two patients and complete lymphadenectomy in one patient. CONCLUSION: COVID-19 vaccine-associated lymphadenopathy is expected to be observed much more frequently in the near future because of increasing vaccination rates. This cause of lymphadenopathy, which may in ultrasound as well as in FDG PET/CT resemble lymph node metastases, must be considered, especially in oncologic patients undergoing tumour follow-up. In addition, COVID-19 vaccination should be given as far away as possible from an underlying primary on the contralateral side to avoid oncologic misdiagnosis followed by malpractice.
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Vacunas contra la COVID-19/efectos adversos , COVID-19/prevención & control , Carcinoma de Células de Merkel/secundario , Ganglios Linfáticos/efectos de los fármacos , Linfadenopatía/inducido químicamente , Melanoma/secundario , Neoplasias Cutáneas/patología , Vacunación/efectos adversos , Adulto , Anciano , Anciano de 80 o más Años , COVID-19/inmunología , COVID-19/virología , Vacunas contra la COVID-19/administración & dosificación , Diagnóstico Diferencial , Errores Diagnósticos , Femenino , Alemania , Humanos , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patología , Linfadenopatía/diagnóstico por imagen , Metástasis Linfática , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Resultado del Tratamiento , UltrasonografíaRESUMEN
BACKGROUND: Metastatic Merkel cell carcinoma (mMCC) has traditionally been managed with palliative chemotherapy regimens or best supportive care (BSC). Avelumab, a novel anti-programmed death-ligand 1 (PD-L1) human monoclonal antibody for mMCC treatment, is being studied in the pivotal JAVELIN Merkel 200 trial. AIM: Incorporating trial results, this analysis aimed to evaluate the cost-utility of avelumab in Taiwan. METHODS AND RESULTS: A de novo partitioned-survival model with three key health states related to survival (progression-free disease, progressed disease, and death) was applied in this study. The data of clinical efficacy, safety, and patient utilities were obtained from the JAVELIN Merkel 200 trial, literature review, and Taiwanese clinical expert opinion. Cost-utility analysis was performed, and results were presented as cost per quality-adjusted life year (QALY) gained. For treatment-naïve patients, the incremental cost-effectiveness ratios (ICERs) for avelumab vs BSC and avelumab vs chemotherapy were US$44885.06 and US$42993.06 per QALY gained, respectively. As to treatment-experienced mMCC patients, avelumab was associated with ICERs of US$27243.06 (vs BSC)/US$26557.43 (vs chemotherapy) per QALY gained. All ICERs remained consistently within the willingness-to-pay (WTP) threshold of US$53,333.33 per QALY gained. CONCLUSION: This study demonstrated avelumab to be a cost-effective treatment option for both treatment-experienced and treatment-naïve mMCC patients with very poor prognosis in Taiwan.
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Anticuerpos Monoclonales Humanizados/economía , Antineoplásicos Inmunológicos/economía , Carcinoma de Células de Merkel/economía , Análisis Costo-Beneficio , Años de Vida Ajustados por Calidad de Vida , Neoplasias Cutáneas/economía , Anticuerpos Monoclonales Humanizados/uso terapéutico , Antineoplásicos Inmunológicos/uso terapéutico , Carcinoma de Células de Merkel/tratamiento farmacológico , Carcinoma de Células de Merkel/secundario , Estudios de Seguimiento , Humanos , Pronóstico , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patología , Tasa de Supervivencia , TaiwánRESUMEN
Aim: To assess clinical outcomes in patients with locally advanced (la) or metastatic (m) Merkel cell carcinoma (MCC) initiating first-line (1L) avelumab in a USA community oncology setting. Materials & methods: Adults with laMCC or mMCC initiating 1L avelumab were identified from The US Oncology Network electronic health record database and chart review. Results: Median overall survival and progression-free survival were not reached in laMCC (n = 9) vs 20.2 and 10.0 months in mMCC (n = 19); response rates were similar (66.7% vs 63.2%). Conclusion: This is the first study to show clinical benefit in patients with laMCC receiving 1L avelumab in a US real-world setting. Response rates in patients with mMCC were consistent with pivotal trials.
Lay abstract Merkel cell carcinoma (MCC) is a rare and aggressive skin cancer. Because MCC progresses quickly, many patients have a poor prognosis. Avelumab is a type of drug that helps the patient's immune system to fight cancer. Avelumab was the first such drug approved by the US FDA for treating metastatic MCC based on the results of the JAVELIN Merkel 200 clinical trial. In SPEAR-Merkel, we studied how MCC patients with locally advanced as well as metastatic disease responded when they were treated with first-line avelumab in a real-world setting. These patients were from oncology practices in communities throughout the USA. Overall response rates in SPEAR-Merkel were comparable between patients with locally advanced and metastatic MCC. Importantly, we found that these patients experienced survival benefit similar to patients in the JAVELIN Merkel 200 (part B) study and other real-world studies.
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Anticuerpos Monoclonales Humanizados/uso terapéutico , Antineoplásicos Inmunológicos/uso terapéutico , Carcinoma de Células de Merkel/tratamiento farmacológico , Neoplasias Cutáneas/tratamiento farmacológico , Anciano , Anciano de 80 o más Años , Carcinoma de Células de Merkel/epidemiología , Carcinoma de Células de Merkel/secundario , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Neoplasias Cutáneas/epidemiología , Neoplasias Cutáneas/patología , Tasa de Supervivencia , Estados Unidos/epidemiologíaRESUMEN
Merkel cell carcinoma (MCC) is a neuroendocrine skin malignancy associated with ultraviolet radiation exposure and the Merkel cell polyomavirus. This study aims to examine associations between primary tumor anatomic site, laterality, and metastases in MCC in the Surveillance Epidemiology and End Results (SEER) database and an academic center MCC database in Rhode Island (RI). A retrospective chart review of 92 MCC cases from RI Hospital and 2845 subjects from the SEER registry was analyzed. Logistic regressions in each cohort were performed to calculate odds ratios (OR), adjusting for sex, age at diagnosis, race, and marital status. The majority of tumors were left-sided in the SEER cohort (53.0%) and RI cohort (56.5%), and located on the head and neck region in the RI cohort (60.9%) and the trunk and extremities in the SEER cohort (70.8%). Left-sided tumors compared to right-sided tumors were marginally associated with increased odds of metastasis in the SEER cohort (OR 1.05, 95% CI 0.87, 1.27); however, decreased risk of MCC was found in the RI cohort (OR 0.33, 95% CI 0.13, 0.80). Tumors located on the trunk and extremities were marginally associated with decreased odds of metastasis compared to head and neck tumors in both databases (SEER: OR 0.88, 95% CI 0.72, 1.08 and RI cohort: OR 0.62, 95% CI 0.25, 1.50). Left-sided tumors located on head and neck showed a suggestive evidence of increased odds of MCC among females in the SEER data (OR 1.77, 95% CI 0.98, 3.23). Our study potentially has important implications for clinical tumor diagnosis and prognostication as well as improved understanding about the epidemiology of MCC.
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Carcinoma de Células de Merkel/epidemiología , Neoplasias Cutáneas/patología , Piel/patología , Anciano , Anciano de 80 o más Años , Carcinoma de Células de Merkel/secundario , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Estudios Retrospectivos , Medición de Riesgo/estadística & datos numéricos , Factores de Riesgo , Programa de VERF/estadística & datos numéricos , Neoplasias Cutáneas/epidemiologíaRESUMEN
OBJECTIVE. Whole-body imaging extending from the vertex of the head to the toes is considered the standard 18F-FDG PET/CT protocol for Merkel cell carcinoma, though the evidence establishing this standard is scant. The purpose of this study was to investigate the clinical impact of PET/CT of the lower extremities in patients with Merkel cell carcinoma, a rare aggressive neuroendocrine tumor of the skin. MATERIALS AND METHODS. A total of 101 patients with Merkel cell carcinoma (mean age, 70.9 years) who underwent whole-body PET/CT were included. PET/CT findings in the lower extremities were evaluated on a per-patient basis, and the results were compared between patients with the primary lesion in the lower extremities (lower extremity primary) and those with the primary lesion located between the head and inguinal regions (body primary). Subsequent clinical evaluation and follow-up imaging were used as the reference standard. RESULTS. In the lower extremity (n = 22) and body (n = 79) primary groups, five and eight patients had true metastases in the lower extremities (p = .15). In the body primary group, all metastases in the lower extremities were part of widespread metastases in the body. In contrast, three of five patients (60%) in the lower extremity primary group had isolated metastases in the lower extremities, which differed significantly from the rate in the body primary group (p = .04). Subgroup analysis that included 48 patients who underwent initial staging examinations showed no metastases in the lower extremities regardless of primary location. CONCLUSION. PET/CT of the lower extremities for patients with body primary lesions of Merkel cell carcinoma should be considered of limited clinical utility.
Asunto(s)
Carcinoma de Células de Merkel/diagnóstico por imagen , Fluorodesoxiglucosa F18 , Extremidad Inferior/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Radiofármacos , Neoplasias Cutáneas/diagnóstico por imagen , Anciano , Carcinoma de Células de Merkel/secundario , Femenino , Humanos , Masculino , Estadificación de Neoplasias/métodos , Estudios Retrospectivos , Sensibilidad y Especificidad , Imagen de Cuerpo EnteroRESUMEN
Oligometastatic disease is a hypothesized intermediate stage of disease between localized and widespread metastatic cancer. Localized treatment of oligometastatic lesions may offer survival advantages in addition to systemic treatment. In this case report, we describe a patient who presented with small cell neuroendocrine carcinoma "Merkel type" (SNECM) of the parotid gland which had metastasized to the brain and adrenal gland. He was treated with chemotherapy followed by stereotactic radiotherapy and volumetric modulated arc therapy for oligometastasis. He maintains good functional status with low burden of disease at 20-months after diagnosis. SNECM is a rare and aggressive parotid cancer with immunohistochemical and morphologic similarities to Merkel cell carcinoma (MCC). There are only 44 cases of parotid SNECM in the English literature. This is the first case to describe management of oligometastatic SNECM and we review literature on management of both SNECM and metastatic MCC.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/secundario , Neoplasias Encefálicas/secundario , Carcinoma de Células de Merkel/secundario , Carcinoma de Células Pequeñas/secundario , Neoplasias de la Parótida/patología , Anciano , Humanos , MasculinoRESUMEN
BACKGROUND: Current lymph node (LN) staging for Merkel cell carcinoma (MCC) does not account for the number of metastatic LNs, which is a primary driver of survival in multiple cancers. OBJECTIVE: To determine the impact of the number of metastatic LNs on survival in MCC. METHODS: Patients with MCC undergoing surgery were identified from the National Cancer Database (NCDB). The association between metastatic LN number and survival was modeled with restricted cubic splines. A novel nodal classification system was derived by using recursive partitioning analysis. MCC patients undergoing surgery in the Surveillance, Epidemiology, and End Results (SEER) Program were used as validation cohort. RESULTS: Among 3670 patients in the NCDB, increasing metastatic LN number was associated with decreased survival (P < .001). Mortality risk increased continuously with each additional positive LN when using multivariable, nonlinear modeling. According to a novel staging system derived via recursive partitioning analysis, the hazard ratio for death in multivariable regression compared with patients without LN involvement was 1.24 (P = .049), 2.08 (P < .001), 3.24 (P < .001), and 6.13 (P < .001) for the proposed N1a (1-3 metastatic LNs with microscopic detection), N1b (1-3 metastatic LNs with macroscopic detection), N2 (4-8 metastatic LNs), and N3 (≥9 metastatic LNs), respectively. This system was validated in the SEER cohort and showed improved concordance compared with the American Joint Committee on Cancer, Eighth Edition. LIMITATIONS: Retrospective design. CONCLUSIONS: Number of metastatic LNs is the dominant nodal factor driving survival in patients with MCC.
Asunto(s)
Carcinoma de Células de Merkel/mortalidad , Escisión del Ganglio Linfático/estadística & datos numéricos , Metástasis Linfática/patología , Neoplasias Cutáneas/mortalidad , Anciano , Anciano de 80 o más Años , Carcinoma de Células de Merkel/diagnóstico , Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/cirugía , Femenino , Humanos , Estimación de Kaplan-Meier , Ganglios Linfáticos/patología , Ganglios Linfáticos/cirugía , Metástasis Linfática/terapia , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Medición de Riesgo/estadística & datos numéricos , Programa de VERF/estadística & datos numéricos , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Carga TumoralAsunto(s)
Neoplasias Óseas/secundario , Neoplasias Encefálicas/secundario , Carcinoma de Células de Merkel/secundario , Neoplasias Hepáticas/secundario , Neoplasias Pulmonares/secundario , Neoplasias Cutáneas/patología , Negro o Afroamericano/estadística & datos numéricos , Factores de Edad , Anciano , Anciano de 80 o más Años , Neoplasias Óseas/mortalidad , Neoplasias Encefálicas/mortalidad , Carcinoma de Células de Merkel/mortalidad , Femenino , Humanos , Estimación de Kaplan-Meier , Neoplasias Hepáticas/mortalidad , Neoplasias Pulmonares/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Programa de VERF/estadística & datos numéricos , Factores Sexuales , Piel/patología , Neoplasias Cutáneas/mortalidad , Estados Unidos/epidemiología , Población Blanca/estadística & datos numéricosRESUMEN
OBJECTIVE: Prognostic factors and optimal treatment approaches for Merkel cell carcinoma (MCC) remain uncertain. This study evaluated the influences of sentinel lymph node (SLN) biopsy and lymphovascular invasion (LVI) on treatment planning and prognosis. STUDY DESIGN: Retrospective cohort study. METHODS: Stage 1 to 3 MCC patients treated 2005 to 2018. Predictors of nodal radiation were tested using logistic regression. Predictors of recurrence-free, disease-specific, and overall survival were tested in Cox proportional hazard models. RESULTS: Of 122 patients, 99 were without clinically apparent nodal metastases. Of these, 76 (77%) underwent excision and SLN biopsy; 29% had metastasis in SLNs, including 20% of MCCs 1 cm or less. Primary tumor diameter, site, patient age, gender, and immunosuppressed status were not significantly associated with an involved SLN. Among patients who underwent SLN biopsy, 13 of 21 (62%) MCCs with LVI had cancer in SLNs compared with 14 of 44 (25.5%) without LVI (P = .003). Although local radiation was common, nodal radiation was infrequently employed in SLN negative (pathologic N0) patients (21.8% vs. 76.2% for patients with SLN metastases, P = .0001). Survival of patients with positive SLNs was unfavorable, regardless of completion lymphadenectomy and/or adjuvant radiation. After accounting for tumor (T) and node (N) classification, age, immunosuppression, and primary site, a positive SLN and LVI were independently associated with worse survival (LVI/recurrence-free survival [RFS]: hazard ratio [HR] 2.3 (1.04-5, P = .04; LVI/disease-specific survival [DSS]: HR 5.2 (1.8-15, P = .007); N1a vs. pN0/RFS HR 3.6 (1.42-9.3, P = .007); DSS HR5.0 (1.3-19, P = .17). CONCLUSION: SLN biopsy assists in risk stratification and radiation treatment planning in MCC. LVI and disease in SLNs, independently associated with worse survival, constitute markers of high-risk disease warranting consideration for investigational studies. LEVEL OF EVIDENCE: III Laryngoscope, 131:E828-E835, 2021.
Asunto(s)
Carcinoma de Células de Merkel/secundario , Carcinoma de Células de Merkel/cirugía , Biopsia del Ganglio Linfático Centinela , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Anciano , Anciano de 80 o más Años , Carcinoma de Células de Merkel/mortalidad , Supervivencia sin Enfermedad , Femenino , Humanos , Modelos Logísticos , Metástasis Linfática , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Estudios Retrospectivos , Neoplasias Cutáneas/mortalidad , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
BACKGROUND: Merkel cell carcinoma (MCC) guidelines derive from melanoma and do not recommend baseline cross-sectional imaging for most patients. However, MCC is more likely to have metastasized at diagnosis than melanoma. OBJECTIVE: To determine how often baseline imaging identifies clinically occult MCC in patients with newly diagnosed disease with and without palpable nodal involvement. METHODS: Analysis of 584 patients with MCC with a cutaneous primary tumor, baseline imaging, no evident distant metastases, and sufficient staging data. RESULTS: Among 492 patients with clinically uninvolved regional nodes, 13.2% had disease upstaged by imaging (8.9% in regional nodes, 4.3% in distant sites). Among 92 patients with clinically involved regional nodes, 10.8% had disease upstaged to distant metastatic disease. Large (>4 cm) and small (<1 cm) primary tumors were both frequently upstaged (29.4% and 7.8%, respectively). Patients who underwent positron emission tomography-computed tomography more often had disease upstaged (16.8% of 352), than those with computed tomography alone (6.9% of 231; P = .0006). LIMITATIONS: This was a retrospective study. CONCLUSIONS: In patients with clinically node-negative disease, baseline imaging showed occult metastatic MCC at a higher rate than reported for melanoma (13.2% vs <1%). Although imaging is already recommended for patients with clinically node-positive MCC, these data suggest that baseline imaging is also indicated for patients with clinically node-negative MCC because upstaging is frequent and markedly alters management and prognosis.