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Skin metastases are rare and may occur in the context of a known metastatic disease or be the first clinical sign of an underlying primary tumor. In the case of carcinoid neoplasms, determining whether the cutaneous tumor is primary or secondary and identifying the tumor origin in metastatic cases is not always an easy task. This is the report of a case of cutaneous metastasis presenting as the first clinical manifestation of a previously unknown pulmonary carcinoid tumor, including the discussion of histopathological and immunohistochemical findings that allowed an adequate diagnosis of the tumor etiology and reinforces the importance for dermatologists and dermatopathologists to be familiar with these findings.

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Background: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. Aim: To review a registry of liver biopsies performed to diagnose hepatic tumors. Patients and Methods: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. Results: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. Conclusions: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

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Background: 85% of neuroendocrine tumors (NET) originate in the gastrointestinal tract, which is why their primary hepatic location is very rare; NETs most frequently cause metastases to the liver; so when diagnosed, a hepatic NET is considered initially metastatic. Diagnosis of primary hepatic neuroendocrine tumors (PHNET) should be performed by excluding extrahepatic NETs and through histological confirmation. The objective of this article is to present a case of PHNET. Clinical case: 75-year-old male patient, who presented asthenia, adynamia, abdominal pain in the right hypochondrium, six-month weight loss, with a right subcostal palpable tumor. Imaging studies reported a lesion in the right hepatic lobe, multilobulated, heterogeneous, with poorly defined margins and with cystic areas. It was performed diagnostic laparotomy and then a hepatic tumorectomy, whose product measured 16.0 x 10.0 x 6.5 cm, with two cystic cavities of 13.2 and 11.5 cm of hematic content. Microscopically, cells with neuroendocrine differentiation, with positive immunoreactivity to chromogranin were observed. It was diagnosed well-differentiated neuroendocrine neoplasm, with cystic degeneration. Conclusions: Even though it is excluded a NET from an extrahepatic primary site, the tumor etiology of an important proportion of patients with PHNET will be due to an unknown primary tumor that will become apparent over time; hence, the need to follow up as long as possible.

Introducción: 85% de los tumores neuroendocrinos (TNE) se originan del tracto gastrointestinal, su localización hepática primaria muy rara; los TNE con mayor frecuencia causan metástasis al hígado; por esto, cuando se diagnóstica un TNE hepático es considerado inicialmente metastásico. El diagnóstico de tumor neuroendocrino primario de hígado (TNEPH) debe realizarse mediante la exclusión de tumores neuroendócrinos extrahepáticos y la confirmación histológica. El objetivo de este artículo es presentar un caso con TNEHP. Caso clínico: paciente de sexo masculino, de 75 años de edad, quien presentó astenia, adinamia, dolor abdominal en hipocondrio derecho y pérdida de peso de 6 meses de evolución, con tumor palpable subcostal derecho. Los estudios de imagen reportaron lesión en lóbulo hepático derecho, multilobulada, márgenes mal definidos, heterogénea y con áreas quísticas. Se realizó laparotomía diagnóstica y tumorectomía hepática que midió 16.0 x10.0 x 6.5 cm, con dos cavidades quísticas de 13.2 y 11.5 cm de contenido hemático. Microscópicamente se observaron células con diferenciación neuroendócrina, inmunoreactivas a cromogranina. Se estableció el diagnóstico de neoplasia neuroendócrina bien diferenciada, con degeneración quística. Conclusiones: aunque se excluya un TNE de sitio primario extrahepático, la etiología tumoral de una proporción considerable de pacientes con TNEPH será a causada por un tumor primario no conocido que se hará evidente con el tiempo; de ahí la necesidad de hacer seguimiento el máximo tiempo posible.

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Large-cell neuroendocrine tumors (NETs) are poorly differentiated malignancies of rare incidence and aggressive nature. NETs mostly arise in the lung followed by the gastrointestinal tract, although they are potentially ubiquitous throughout the body. Primary unknown NET has a worse prognosis and shorter survival comparing with other NETs, with limited available data in the literature concerning this subgroup. The authors report the case of large-cell NET with supraclavicular lymph node presentation. Total excisional biopsy revealed an enlarged adenopathy 18 × 15 × 10 mm, which was extensively infiltrated by a solid malignant neoplasm composed of large cells with granular chromatin, nuclear pseudo-inclusions, high mitotic index, and focal necrosis, with a Ki 67 index 25-30% and positive immunohistochemical study for the expression of cytokeratin 8/18, chromogranin, synaptophysin, and thyroid transcriptional factor-1 (TTF-1). There was no evidence of primary location apart from two infracentimetric lung lesions that could not be accessed for biopsy and were negative at both somatostatin receptor scintigraphy and positron emission tomography. The NET relapsed with three mediastinal masses, so the patient was started on chemotherapy with carboplatin and etoposide with initial total response. Early progression showed no response to further chemotherapy regimens (temozolomide, oral etoposide); therefore, the patient was treated with local radiotherapy. This patient has an atypical long survival (54 months) compared to the literature data. In fact, there are few long-term survivors of large-cell NET and they are all related to complete surgical resection.

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Large-cell neuroendocrine tumors (NETs) are poorly differentiated malignancies of rare incidence and aggressive nature. NETs mostly arise in the lung followed by the gastrointestinal tract, although they are potentially ubiquitous throughout the body. Primary unknown NET has a worse prognosis and shorter survival comparing with other NETs, with limited available data in the literature concerning this subgroup. The authors report the case of large-cell NET with supraclavicular lymph node presentation. Total excisional biopsy revealed an enlarged adenopathy 18 × 15 × 10 mm, which was extensively infiltrated by a solid malignant neoplasm composed of large cells with granular chromatin, nuclear pseudo-inclusions, high mitotic index, and focal necrosis, with a Ki 67 index 25-30% and positive immunohistochemical study for the expression of cytokeratin 8/18, chromogranin, synaptophysin, and thyroid transcriptional factor-1 (TTF-1). There was no evidence of primary location apart from two infracentimetric lung lesions that could not be accessed for biopsy and were negative at both somatostatin receptor scintigraphy and positron emission tomography. The NET relapsed with three mediastinal masses, so the patient was started on chemotherapy with carboplatin and etoposide with initial total response. Early progression showed no response to further chemotherapy regimens (temozolomide, oral etoposide); therefore, the patient was treated with local radiotherapy. This patient has an atypical long survival (54 months) compared to the literature data. In fact, there are few long-term survivors of large-cell NET and they are all related to complete surgical resection.

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Abstract: On rare occasions, skin lesions are the first local of metastatic manifestation of internal malignancies. In case of no previous diagnosis of these tumors, the approach of suspicious skin lesions becomes a challenge, especially in differentiating cutaneous metastases and adnexal primary neoplasms. Currently, besides epidemiologic, dermoscopic and histopathologic aspects, the evaluation also integrates immunohistochemical exams and cell markers such as p40 and p63, highly specific for skin metastases. This article describes the case of cutaneous metastases as the sole obvious sign of breast cancer in a previously asymptomatic woman. The diagnosis was made by the finding of neoplastic cells in the dermis and immunohistochemistry compatible with ductal carcinoma.

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Abstract Metastatic ovarian cancer uncommonly presents with skin metastasis. When present, skin metastases of ovarian cancer are usually localized in the vicinity of the primary tumor. We report a case of a 58-year-old woman with a rapid growing erythematous, well-defined nodule localized on the left nasal ala. A skin biopsy was performed and histopathological and immunohistochemical findings were compatible with a cutaneous metastasis of adenocarcinoma. A systematic investigation revealed a bilateral ovarian cystadenocarcinoma associated with visceral dissemination, likely associated with nose cutaneous metastasis. We report a very uncommon case because of the presentation of ovarian carcinoma as cutaneous metastasis. To our knowledge, this atypical localization on the nose has not been described yet in the literature.

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Abstract: We describe herein what is to our knowledge the first reported case of an invasive cutaneous metastasis with unknown primary, electively treated solely with electrochemotherapy. We describe a female patient with a large, invasive and painful lesion in her hypogastric region, extending up to the pubic area. The cutaneous biopsy and instrumental and laboratory analyses, all failed to reveal the primary site. A final diagnosis of cutaneous metastasis with unknown primary was made and treatment was performed with electrochemotherapy. Our case highlights the importance of interdisciplinary choices in clinical practice to cope with the lack of a primary site and to improve quality of life, since no standardized therapy exists for these classes of patients.

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AbstractCutaneous metastasis is a rare manifestation of visceral malignancies that indicates primarily advanced disease. Due to its low incidence and similarity to other cutaneous lesions, it is not uncommon to have a delayed diagnosis and a shortened prognosis. We describe the case of a patient who presented with a cutaneous nodule in the sternal region as a first sign of malignancy.

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Introdução: O melanoma é essencialmente cutâneo. Em alguns pacientes, não é possível determinar a localização do tumor primário. A incidência de melanoma com sítio primário desconhecido varia de 2 a 15%. Objetivos: Determinar se há diferença de sobrevida global entre os pacientes com melanoma primário conhecido comparado aos pacientes com melanoma primário desconhecido. Métodos: Foi realizada análise retrospectiva de pacientes com melanoma no banco de dados da Oncologia Cirúrgica e Cirurgia do Aparelho Digestivo (ONCAD) e identificados aqueles com sítio primário desconhecido e metástases para diferentes locais. Resultados: O principal local de metástases foi o linfonodo (50%) - inguinais (25%),axilares (16%) e periaórticos (8,3%). Metástases pulmonares foram encontradas em três pacientes (25%). Metástases para fígado, osso e pele foram observadas em um caso cada (8,3%). Conclusão: A evolução clínica dos pacientes metastáticos com melanoma de sítio primário desconhecido é melhor em relação aos pacientes metastáticos com lesão primária conhecida, quando os dois grupos estão no mesmo estádio. Dessa forma, o fator mais determinante do curso clínico e do prognóstico éa localização das metástases. A maioria dos pacientes que apresenta doença sistêmica ao diagnóstico perde a chance de cura, como muitos pacientes com cutâneo primário fino e doença regional ao diagnóstico.

Introduction: Melanoma is essentially cutaneous. In some patients, it is not possible to determine the location of the primary tumor. The incidence of melanoma of unknown primary site varies from 2 ? 15%. Objectives: To determine whether there is difference in overall survival between patients with known primary melanoma compared to patients with unknown primary melanoma. Methods: A retrospective analysis of patients with melanoma in the database of Surgical Oncology and Digestive Surgery (ONCAD) was performed and identified those with unknown primary site and metastases to different locations. Results: Lymph node was the main site of metastases (50%) - inguinal (25%), axillary (16%) and periaortic (8.3%). Pulmonary metastases were found in three patients ( 25%). Metastasis to the liver, bone and skin were observed in one case each (8.3%). Conclusions: The clinical course of patients with metastatic melanomaof unknown primary site is better than metastatic patients with known primary lesion, when both groups are on the same stage. Thus, the most relevant determining factor affecting clinical course and prognosis is the metastasis location. Most patients presenting systemic disease at diagnosis loses the chance of cure as many patients with thin primary cutaneous and regional disease at diagnosis.

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BACKGROUND: While representing only 3-4% of malignant skin tumors, cutaneous melanoma is the most aggressive and lethal. Statistical knowledge about the biological behavior of this tumor is essential for guiding daily outpatient practice and aiding public health policies. OBJECTIVES: To analyze the profile of patients with cutaneous melanoma attending a pathology department in Teresina (state of Piauí) between 2000 and 2010. METHODS: Retrospective study of melanoma patients diagnosed between 2000 and 2010 in the São Marcos Hospital in the city of Teresina. The pathology laboratory reports were studied and all the statistical analyses performed using SPSS 19.0. RESULTS: A total of 25 in situ, 199 invasive and 89 metastatic melanomas of unknown primary site were observed. Histological types found were nodular (52.8%), superficial spreading melanoma (18.6%), acral (10.6%) and lentigo maligna (9.5%). In 144 (73.4%) cases the Breslow thickness was >1 mm. Metastasis was found in 28.6% of invasive melanomas and nodular melanoma, Clark IV/ V, Breslow > 1 mm, mitotic index > 6 and ulcerated lesions were more likely to metastasize. CONCLUSION: Most melanomas presented Breslow> 1mm. The main factors associated with metastasis were nodular type, Clark IV / V, Breslow> 1mm, mitotic index > 6 and ulcerated lesions. .

FUNDAMENTOS: Apesar de representar apenas 3-4% dos tumores malignos de pele, o melanoma cutâneo é o mais agressivo e letal deles. O conhecimento estatístico do comportamento biológico deste tumor em nosso meio ambiente é fundamental para orientar a prática ambulatorial diária e para auxiliar políticas de saúde pública. OBJETIVOS: Analisar o perfil de pacientes com melanoma cutâneo diagnosticados em serviço de referência em patologia em Teresina-Piauí no período de 2000 a 2010. MÉTODOS: Estudo retrospectivo de pacientes com melanoma diagnosticados entre 2000 e 2010 no Hospital São Marcos, Teresina-Piauí-Brasil. Estudou-se laudos histopatológicos e realizou-se análises estatísticas com o programa SPSS 19,0. RESULTADOS: Um total de 25 melanomas in situ, 199 invasivos e 89 metastáticos de sítio primário desconhecido foram observados. Tipos histológicos encontrados foram nodular (52,8%), melanoma extensivo superficial (18,6%), acral (10,6%) e lentigo maligno (9,5%). Em 144 (73,4%) casos o índice de Breslow foi >1 mm. Verificou-se metástases em 28,6% dos melanomas invasivos e melanoma nodular, Clark IV/V, Breslow >1 mm, índice mitótico ≥6 e lesões ulceradas estavam mais propensos a metástases. CONCLUSÃO: Melanomas com Breslow >1mm foram os casos predominantes. Principais fatores ...

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The malignant melanoma is a relatively common neoplasia, with origin generally in the melanocytics cells in the skin, but with presentation of other possible primary lesions, being presented in this, a case witnessed of liver and mesentery metastases with unknown primary sites.

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Since the 19th century, neuroendocrine neoplasms (NEN) have been identified. Right up to the present day, the nomenclature is still under debate reflecting the heterogeneity of these tumors. Although some of them are slow growing, some can be fearfully aggressive and may develop in almost any organ of the abdomen, thorax, neck, skin, and gonads. The most commonly observed sites of NEN are the lung and the gastroenteropancreatic system (GEP), where more than 50 entities have been observed. In case of a NEN of unknown primary tumor, the histopathological diagnostic workup includes immunohistochemistry for chromogranin A and synaptophysin, followed by specific tissue markers. Clinical presentation is very diverse, depending on the primary site and functionality of the tumor. In the case of the GEP-NEN, the main symptoms are abdominal pain, diarrhea, weight loss, gastrointestinal bleeding, or bowel obstruction. The presence of neuropsychiatric symptoms is not insignificant in this group of tumors. The authors report a case of a 51-year-old man who sought medical attention because of a three-month history of a consumptive disease. The diagnostic workup disclosed a diffuse nodular infiltration of the lungs, hypokalemia, and hypercalcemia in a cachectic patient. The clinical investigation could not proceed because of an infectious intercurrence, which led to the fatal outcome. Autopsy findings showed a diffuse metastatic NEN. The primary tumoral site could not be demonstrated with the available immunohistochemical panel.