Collagenous sprue: a rare cause of watery diarrhea and villous atrophy - case report.

Collagenous sprue is a rare and unrecognized cause of diarrhea and weight loss, mainly affecting the duodenum and small bowel. The clinical picture often resembles that of coeliac sprue, the main differential diagnosis, albeit, being refractory to a gluten-free diet. The histological features are fundamentally characterized by the deposition of collagen beneath the basement membrane of gut mucosa. Treatment should be initiated as soon as the diagnosis is established, so as to prevent the progression of fibrosis. We will describe the case of a 76-year-old woman with collagenous sprue, her diagnostic workup, histopathological examination, and response to treatment.

Invasive Lobular Carcinoma Arising in Ectopic Breast Tissue: A Case Report.

Incomplete regression of the embryonic mammary line occurs in 0.3-6% of the population. Ectopic breast tissue is mostly asymptomatic and can undergo malignant transformation. Ectopic breast cancer accounts for 0.2-0.6% of all breast cancers. Screening breast examinations can miss these lesions due to their location making the diagnosis more challenging. We describe a case of a primary invasive lobular carcinoma in an ectopic breast on the left axilla detected in a 49-year-old woman. Firstly diagnosed as a sebaceous cyst, the lesion was excised under local anesthesia. Histopathology showed breast tissue widely infiltrated by an invasive carcinoma. Excision of the remnant tissue with axillary lymph node dissection was performed. Ectopic breast carcinoma is a rare diagnosis and there is a general lack of awareness. The presence of an abnormal mass along the mammary ridge should raise clinicians' attention. Management of primary ectopic breast carcinoma should be based on a multidisciplinary approach under the same principles as breast cancer. Furthermore, it does not appear to bring a worse prognosis when diagnosed at similar disease stages.

An Atypical Pleomorphic Lipomatous Tumor Presenting As Groin Mass.

Cutaneous tumors with adipocyte differentiation are frequently excised by surgeons in their daily clinical practice and sometimes less common histological diagnoses arise. Knowledge of different pathological entities and their natural history is essential for better patient management. Atypical spindle cell/Pleomorphic lipomatous tumor (ASPLT) is a recent group included in the WHO classification. We report a case of a middle-aged man with an atypical pleomorphic lipomatous tumor in an unusual location.

A Case of Cervical Lymphadenopathy After Vaccination Against COVID-19.

The coronavirus disease 2019 (COVID-19) pandemic has caused a major global healthcare crisis, and the fields of science and medicine have been engaged in a massive effort to control and prevent the resultant deaths and morbidity. Researchers and pharmaceutical companies have developed in record time vaccines against COVID-19 that are intended to be safe and effective; however, the short validation time has been a challenge for doctors and epidemiologists, especially in light of the increase in reports emerging from various parts of the world about the adverse effects of the new vaccines. Portugal's national regulatory authority, the National Authority of Medicines and Health Products (INFARMED), has recently granted approval for Pfizer-BioNTech (Pfizer Inc., New York, NY; BioNTech SE, Mainz, Germany) and Moderna (Moderna, Inc, Cambridge, MA) COVID-19 vaccines, and they are being rolled out to be administered among the general population. In light of this, it is important for breast surgeons, family doctors, hematologists, and radiologists to consider the effects of recent COVID-19 vaccination history as a possible cause in the differential diagnosis for patients with unilateral cervical adenopathy. The objective of this report is to present a case that involves an adverse reaction involving acute-onset cervical lymphadenopathy in a female patient that coincided with her vaccination against COVID-19, even though cervical lymphadenopathy had not been previously reported as a potential side effect of the COVID-19 vaccination. We discuss the case of a Portuguese physician with a family history of breast cancer, who developed right cervical lymphadenopathy after receiving the first dose of the COVID-19 vaccine. Lymph node growth and ultrasound changes observed in the patient over the weeks, and a lack of information on the COVID-19 vaccine's adverse effects, prompted an in-depth study to understand its etiology.

Unusual Cutaneous Metastases From Colon Adenocarcinoma.

Skin metastases from internal malignancies are rare, even more from colorectal tumours, and are usually a sign of disseminated disease with a poor prognosis. However, with increased numbers of cancer survivors, a high index of suspicion should exist during the evaluation of cutaneous lesions since it can be the initial sign of disease progression or even the first sign that triggers a malignancy diagnosis, potentially improving the patient's odds. Herein, we report a case of synchronous cutaneous metastases from colon adenocarcinoma with unusual localization on the back and forearm of a 65-year-old man.

Peripheral neuropathy in systemic vasculitis and other autoimmune diseases - a report of five cases emphasizing the importance of etiologic characterization.

INTRODUCTION: Peripheral neuropathies may present in the context of systemic vasculitis and other autoimmune diseases. The etiologic characterization is crucial to define the treatment and prognosis in secondary vasculitis. The purpose of this study is to describe the pathway of etiologic investigation including the role of nerve biopsy. METHODS: Retrospective analysis of patients seen in the neuromuscular outpatient clinic during the last four years with peripheral neuropathy in the context of systemic vasculitis or other autoimmune diseases. RESULTS: We present five patients with stepwise progressive sensorimotor deficits of upper and lower limbs. All patients presented with systemic features and one of them had an established diagnosis of systemic vasculitis. They underwent an extended blood panel, including autoimmune and serologic tests. Electromyography and nerve conduction studies revealed asymmetric axonal sensorimotor polyneuropathies in four patients, and an axonal sensorimotor multiple mononeuropathy in one. Four patients underwent nerve biopsy and the other performed a skin biopsy, with findings suggestive of possible vasculitic processes. The etiologies identified included microscopic polyangiitis, HBV-related polyarteritis nodosa and two eosinophilic granulomatosis with polyangiitis. In the last patient a specific etiology could not be established. CONCLUSION: This series reveals the etiologic and phenotypic diversity of peripheral neuropathies related with systemic vasculitis. The therapeutic approach and prognosis were distinct in each patient, emphasizing the importance of a prompt diagnosis and appropriate treatment.

Large mucinous cystic neoplasm of the pancreas during pregnancy: a case report.

Mucinous cystic neoplasms (MCNs) of the pancreas occurring during pregnancy constitute a rare condition requiring special management due to its large size, malignant potential and the risk of fetal growth restriction. The key points to manage MCNs are to perform a correct diagnosis, accurately evaluate the grade of malignant potential in order to establish the right time for pancreatic surgery and to choose the most appropriate fetal mode of delivery. We describe a case of an incidental abdominal mass detected in a third-trimester pregnant woman. At surgery, a huge pancreatic cystic tumour was found and a distal pancreatectomy with splenic preservation was performed. The histopathological analysis revealed an MCN of the pancreas with low-grade dysplasia. To our knowledge, this is the largest pancreatic MCN detected in a pregnant woman reported in the literature and one of the few successfully resected after vaginal delivery.

Intraplacental Choriocarcinoma: Rare or Underdiagnosed? Report of 2 Cases Diagnosed after an Incomplete Miscarriage and a Preterm Spontaneous Vaginal Delivery.

Intraplacental choriocarcinoma is a rare malignant tumor diagnosed after an abortion, an ectopic pregnancy, or a term or preterm pregnancy or following the diagnosis of a hydatidiform mole. During pregnancy, it may be more common than reported, as most patients are asymptomatic and placental choriocarcinomas are usually inconspicuous macroscopically and are often mistaken for an infarct. Based upon a case study methodology, we describe 2 cases of intraplacental choriocarcinoma: the first case was identified in the product of a uterine curettage following an incomplete miscarriage and the second in one of the placentas of a bichorionic twin pregnancy. Maternal investigation did not reveal evidence of metastatic disease and neither did the infants' one in the second case. The two cases underwent maternal surveillance with serum hCG and remained disease-free until the present. In conclusion, intraplacental choriocarcinoma is easily underdiagnosed but with current treatment, even in the presence of metastasis, the prognosis is excellent. A routine microscopic examination of all the placentas and products of miscarriage can increase the real incidence of this entity and consequently improve its management.

Adult pancreatoblastoma - Case report and review of literature.

Most cases of pancreatoblastoma, a rare malignant epithelial tumor of the pancreas, are seen in the pediatric population. The rarity of pancreatoblastoma, the similar radiologic findings to those seen in other pancreatic lesions, and its histopathologic heterogeneity, make its preoperative diagnosis in adults a real challenge. We report ultrasound, computed tomography and magnetic resonance imaging correlative findings of a histologically proven pancreatoblastoma in a 37-year-old woman. Pancreatoblastoma should be considered in the differential diagnosis of a pancreatic mass presenting uncommon imaging features.

Neuropatía atípica asociada a microvasculitis positiva para ANCA-MPO / Atypical neuropathy in MPO-ANCA small vessel vasculitis

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Solid pseudopapillary tumor of the pancreas: a rare and probably misdiagnosed neoplasm.

Solid Pseudopapillary Tumor of the pancreas is a rare nonfunctioning tumor. It is considered a low-grade malignancy that is apparently curable with surgical complete excision in most instances. We present a case of solid pseudopapillary pancreatic tumor that represented a challenge to the radiologists. This case highlights its possible various appearances and the need to the radiologist to be familiar with them.

Malformaçäo urológica em criança com síndrome de Sotos: relato de caso / Urological anomaly in an infant with Sotos' syndrome: report of a case

Objetivo: Abordar o diagnótico e as consideraçöes etiológicas de malformaçäo urológica bilateral em um caso de síndrome de Sotos (gigantismo cerebral). Alertar os pediatras e urologistas da existência dessa associçäo lesional. Criança de 3 meses de idade nascida com 35 semanas através de cesariana de mäe hígida, com as características fenotípicas e perfil metacarpofalâgica de síndrome de Sotos, apresentou taquipnéia, taquicardia e episodios recorrentes de febre (ate 36§ C). A urocultura mostrou 500.000 col/ml. A uretocistografia mostrou megaureters e dilataçäo calicial de ambos os rins. Com diagnóstico de refluxo vésico-uretral grau IV bilateral, foi tratada com terapia antimicrobiana. A evoluçäo foi a melhoria progressiva e obteve alta hospitalar no momento em que se apresentou assintomática, recebendo nitrofurantoína com profilaxia. Assim como em outros casos de infecçäo do trato urinário, os pediatras devem suspeitar de anomalias urológicas em todas as crianças com gigantismo cerebral que apresentarem quadro clínico de síndrome de Sotos. Deficiência embriológica da inervaçäo do sistema nervoso autônomo na musculatura dos ureteres poderiam ser uma outra causa

Carcinoma epidermôide primário da glândula mamária / Primary squamous cell carcinoma of the breast

Os autores apresentam um caso extremamente raro de carcinoma espinocelular primário da glândula mamária direita. Tratava-se de uma paciente de 41 anos, com antecedentes cirúrgicos há 1 ano e meio de papiloma intraductal e mastite crônica, que referia lesäo tipo abscesso, que drenava material hemopurulento. O processo piorou, e apalpou-se nódulo profundo, cujo diagnóstico anátomo-patológico após cirurgia foi carcinoma epidermóide bem diferenciado. Apesar do tratamento agressivo, a evoluçäo foi rapidamente mortal. A histogênese, assim como os diagnósticos diferenciados, é discutida

Progressive premalignant and malignant development (metachronous appearance) in multiple burn scars (Marjolin's ulcers): report of a rare case and review of the literature

O epônimo úlcera de Marjolin refere-se à transformação maligna de qualquer úlcera ou cicatriz da pele. Relata-se o caso de uma mulher, branca, de 35 anos de idade, que sofreu queimadura térmica na metade inferior do seu corpo quando estava com 8 anos de idade. Após 16 anos, e ao longo de 9 anos, apareceram em forma metácrona na pele de ambas as coxas, úlceras, lesões de hiperplasia pseudocarcinomatosa e carcinomas epidermóides. Discute-se a história clínica, a histopatologia e histogênese, o comportamento biológico, assim como o tratamento. A literatura mais relevante foi revisada.

Tumores carcinóides mucinosos da glândula mamária / Mucinous carcinoid tumors of the breast

Na avaliaçäo retrospectiva de 28 pacientes lobulares mamários, os autores encontram três casos de carcinoma neuroendócrino com diferenciaçäo mucinosa (também conhecidos como carcinóide mucossecretante). Tratavam-se de mulheres jovens (média de 40,6 anos) com tumores nos quadrantes laterais da glândula mamária direita. Os tumores tinham aspecto sólido ou cístico-gelatinoso, diâmetro médio de 30 mm e margens bem delimitadas. Na histopatologia, o padräo basalóide foi o mais freqüente, com acúmulo de células sem atipias nem mitoses, apresentando argirofilia positiva, além da presença de mucina extracelular em porcentagens variável, nunca superior a 33 por cento. Nenhum dos casos, apresentou metástases axilares nem à distância, e näo foi observada síndrome carcinóide. Discute-se a histogênese, o comportamento biológico e o tratamento dessa neoplasia

Tumor carcinóide neuroendócrino da mama: estudo clínico-patológico de seis casos / Neuroendocrine carcinoid tumors of breast: clinicopathologic study of six cases

O tumor carcinóide primário da glândula mamária é uma neoplasia muito rara, de existência controversa. Sua histogênese ainda é discutida, sendo a origem mais reconhecida as células basais primitivas ductais. No estudo retrospectivo de 243 carcinomas infiltrativos e "in situ" de tipo ductal e lobular, seguindo estritos critérios de seleçäo, foram achados seis tumores carcinóides. O material correspondia a mulheres com idade média de 43 anos, que apresentaram-se com massas palpáveis notadamente nos quadrantes externos da glândula mamária direita. Näo foram comprovadas metástases axilares nem síndrome carcinóide. A mamografia mostrou opacidades densas de contornos regulares. A punçäo citológica com agulha fina sugeriu células neoplásicas de tipo lobular em três casos; hiperplasia lobular atípica em um caso e presença de mucina näo específica no resto. No estádio II da doença foi realizada mastectomia de tipo Madden em todos os casos. Macroscopicamente os tumores tinham uma média de 29,8 mm de diâmetro, aspecto sólido em quatro casos e cístico-gelatinoso em dois, e margens bem definidas. O padräo histopatológico mais freqüente foi o basalóide e presença de mucina extracelular compondo 28 porcento do tumor em três casos. Näo foram comprovadas mitoses. Os diagnósticos diferenciais incluíram: o carcinoma lobular, o carcinoma mucinoso e as metástases mamárias de carcinóides de outras topografias. Na evoluçäo pós-operatória näo foi comprovada recorrência local, disseminaçäo a distância ou óbito numa média de 3,8 anos, sendo que em nenhuma das pacientes realizou-se rádio, químio ou hormonioterapia. Baseados nesses dados propöe-se a quadrantectomia com esvaziamento axilar como primeira forma de tratamento, reservando a mastectomia para um segundo momento.

Endometriosis colónica: consideraciones diagnósticas y terapéuticas / Endometriosis of the colon: diagnostic and therapeutic considerations

Se presentan dos casos de endometriosis colónica. Se señalan sus aspectos etiopatogénicos, anatomopatológicos, clínicos y los exámenes complementarios más importantes. En cuanto al tratamiento se destaca la necesidad de resección intestinal frente a la endometriosis colónica sintomática