Epithelioid pituicytoma: An unusual case report.

BACKGROUND: Pituicytomas are considered World Health Organization Grade I malignancies. Until September 2017, a total of 81 cases of pituicytomas were diagnosed and described in literature. We present such a case in which histopathology shows an epithelioid pattern, a rare variant of pituicytoma. As far as we know, this is only the second such case described in the literature. CASE DESCRIPTION: A 61-year-old male patient presented with complaints of progressive decrease in visual acuity for about 7 months, worse on the left side. Laboratory and endocrinological investigation returned normal values. Magnetic resonance imaging revealed a mixed solid-cystic lesion, measuring about 3.1 × 2.2 × 2.9 cm. The lesion presented with intermediate signal intensity in T1 and T2 sequences and showed avid postcontrast enhancement. The patient underwent resection through a left pterional approach. Pathology revealed a glial neoplasm with an epithelioid pattern and moderate cellularity with rounded-elongated cell nuclei and with a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, any mitotic figures, or areas of necrosis was noted. CONCLUSION: The epithelioid variant of pituicytomas differs from the commonly encountered forms of this tumor which typically present in a fascicular pattern. Microsurgical resection is the treatment of choice. However, in many cases, subtotal resection was performed because of a considerable risk for neurovascular injuries.

Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma.

INTRODUCTION: Chordomas are rare malignancies of bone origin that occur in the axial skeleton, typically the skull base and lumbar/sacral regions. Although often classified as low-grade neoplasms, its locally infiltrative behavior may result in significant morbidity and mortality. Optimal surgical resection may be curative, but up to 50% of the cases relapse within 5 years, and currently there are no systemic treatments approved in this setting. A large proportion of these tumors express stem-cell factor receptor (c-KIT) and platelet-derived growth factor receptors (PDGFRs), providing a rationale for the use of tyrosine-kinase inhibitors (TKIs). CASE REPORT: A 27-year-old male presented with recurrent chordoma of the lumbar spine 4 years after initial diagnosis. Salvage therapies in the interval included repeat resections and radiation therapy. He ultimately developed multifocal recurrence not amenable to complete excision or reirradiation. A comprehensive genomic profiling assay was performed and revealed nondrugable alterations. Decision was made to proceed with systemic treatment with pazopanib 800 mg/day, resulting in tumor reduction (-23.1% reduction in size) and prolonged disease control. CONCLUSION: For this patient with a multiple recurrent chordoma and limited treatment options, pazopanib resulted in sustained clinical benefit following initial tumor reduction.

Low-grade Endometrial Stromal Sarcoma with Unusual Metastasis to the Skull

Background Endometrial stromal sarcomas (ESSs) are the second most common uterine sarcomas. Although ESSs are often indolent, they have metastatic potential. To the best of our knowledge, there are only three reports of brain metastasis, and the present report is the first to describe a late skull metastasis of an ESS. Case Report We describe the case of a 51-year-old woman who presented abnormal vaginal bleeding 14 years ago; she was diagnosed with an uterine mass and submitted to a hysterectomy. One year ago she presented ESS lung metastasis followed by a left parietal calvarial metastasis. The optimal treatment for metastatic ESS is controversial, but the use of progesterone and aromatase inhibitors is advisable.

Introdução Sarcoma endometrial estromal (SEE) é a segunda lesão mais frequente dentre os sarcomas uterinos. Geralmente são lesões indolentes, mas com potencial de desenvolver metástase. Até o momento há apenas três relatos de metástase cerebral, sendo este o primeiro estudo a descrever uma metástase craniana tardia dessas lesões. Relato de caso Nós descrevemos o caso de uma paciente de 51 anos de idade que apresentou há 14 anos um quadro de sangramento vaginal anormal, sendo diagnosticada uma massa uterina; a paciente foi submetida a uma histerectomia. Há um ano ela evoluiu com metástase pulmonar, seguida por metástase craniana parietal esquerda. O tratamento ideal do SEE metastático ainda é controverso, mas o uso de inibidores de aromatase é aconselhável.

Eosinophilic Solid and Cystic Renal Cell Carcinoma: Imaging Features of a Novel Neoplasm.

Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a recently described entity with distinct clinical, pathologic, and molecular features. However, the radiological aspects of ESC RCC have not been characterized. In this report, we describe the imaging findings of 2 ESC RCCs. We found 2 distinct imaging patterns that varied depending on histopathologic features (solid or cystic predominance). In conclusion, it is important to know the imaging characteristics and pathologic correlation of this novel neoplasm to increase its recognition and to improve the decision-making process.

Functional Reconstruction of Temporomandibular Joint after Resection of Pigmented Villonodular Synovitis with Extension to Infratemporal Fossa and Skull Base: A Case Report.

Introduction Pigmented villonodular synovitis (PVNS) is a benign but aggressive lesion arising from sinovia. The temporomandibular joint (TMJ) is hardly ever involved. Methods We describe a case of PVNS arising in the left TMJ involving infratemporal fossa soft tissue and the skull base; we also present the reconstruction. Results A 37-year-old woman had progressive mandibular swelling for 6 months. Computed tomography of the skull revealed an osteolytic lesion in the left TMJ, involving the upper mandible, condyle, and glenoid fossa and extending to the infratemporal fossa and fossa media through a defect in temporal bone. Surgical management included a left pterional craniotomy to reach the temporal skull base and resect the intracranial tumor and a facial approach with partial left mandibulectomy and resection of left condyle, glenoid fossa, and tumor removal in infratemporal fossa. Mandible function was restored with prosthetic reconstruction of the condyle. She progressively started to eat solid foods after 3 months, becoming increasingly functional and asymptomatic. At 30 months' follow-up, she had no sign of tumoral recurrence and showed asymptomatic and normal TMJ function. Conclusion PVNS should be considered in the differential diagnosis of bone neoplasms affecting young patients. In such cases, radical excision is mandatory and TMJ prosthesis for local reconstruction may be used to preserve functionality.

Plasmacytoid urothelial carcinoma: a case of histological variant of urinary bladder cancer with aggressive behavior.

Plasmocytoid variant urothelial carcinoma (PVUC) of the urinary bladder is a rare histological variant of transitional cell carcinoma. Data regarding PVUC shows that this neoplasia presents a distinctive clinical outcome represented by aggressive behavior and poor survival rate. The authors report a case of a 57-year-old male patient with a 3-month history of hematuria and pelvic pain. Radical cystectomy with lymphadenectomy was performed and pathological examination showed a pT3pN0 PVUC of the bladder. The patient remained free of recurrence for 8 months, but the disease recurred involving the abdominal wall and subcutaneous tissue. Chemotherapy provided a positive clinical response and relief of symptoms. The authors call attention to the aggressiveness of this rare variant of bladder cancer and recommend radical surgery and multidisciplinary management of this neoplasm.

Clinically low-risk prostate cancer: evaluation with transrectal doppler ultrasound and functional magnetic resonance imaging.

OBJECTIVES: To evaluate transrectal ultrasound, amplitude Doppler ultrasound, conventional T2-weighted magnetic resonance imaging, spectroscopy and dynamic contrast-enhanced magnetic resonance imaging in localizing and locally staging low-risk prostate cancer. INTRODUCTION: Prostate cancer has been diagnosed at earlier stages and the most accepted classification for low-risk prostate cancer is based on clinical stage T1c or T2a, Gleason score ≤6, and prostate-specific antigen (PSA) ≤10 ng/ml. METHODS: From 2005 to 2006, magnetic resonance imaging was performed in 42 patients, and transrectal ultrasound in 26 of these patients. Seven patients were excluded from the study. Mean patient age was 64.94 years and mean serum PSA was 6.05 ng/ml. The examinations were analyzed for tumor identification and location in prostate sextants, detection of extracapsular extension, and seminal vesicle invasion, using surgical pathology findings as the gold standard. RESULTS: Sixteen patients (45.7%) had pathologically proven organ-confined disease, 11 (31.4%) had positive surgical margin, 8 (28.9%) had extracapsular extension, and 3 (8.6%) presented with extracapsular extension and seminal vesicle invasion. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy values for localizing low-risk prostate cancer were 53.1%, 48.3%, 63.4%, 37.8% and 51.3% for transrectal ultrasound; 70.4%, 36.2%, 65.1%, 42.0% and 57.7% for amplitude Doppler ultrasound; 71.5%, 58.9%, 76.6%, 52.4% and 67.1% for magnetic resonance imaging; 70.4%, 58.7%, 78.4%, 48.2% and 66.7% for magnetic resonance spectroscopy; 67.2%, 65.7%, 79.3%, 50.6% and 66.7% for dynamic contrast-enhanced magnetic resonance imaging, respectively. Sensitivity, specificity, PPV, NPV and accuracy values for detecting extracapsular extension were 33.3%, 92%, 14.3%, 97.2% and 89.7% for transrectal ultrasound and 50.0%, 77.6%, 13.7%, 95.6% and 75.7% for magnetic resonance imaging, respectively. For detecting seminal vesicle invasion, these values were 66.7%, 85.7%, 22.2%, 97.7% and 84.6% for transrectal ultrasound and 40.0%, 83.1%, 15.4%, 94.7% and 80.0% for magnetic resonance imaging. CONCLUSION: Although preliminary, our results suggest that imaging modalities have limited usefulness in localizing and locally staging clinically low-risk prostate cancer.

Carcinomas uroteliais de bexiga: aspectos anatomopatológicos e imuno-histoquímicos. Pesquisa de metaloproteinases de matriz utilizando a técnica de tissue microarray (TMA) / Urothelial carcinomas of the urinary bladder: morphological and immunohistochemical aspects. Expression of the matrix metalloproteinases using the tissue microarray (TMA) technique

OBJETIVOS: Estudar variáveis anatomopatológicas relacionadas à progressão tumoral em carcinomas uroteliais primários de bexiga e sua associação com a imunoexpressão de metaloproteinases de matriz (MMPs) - 2, -9 e -14 no epitélio e no estroma dos tumores primários e nas metástases linfonodais. MÉTODOS: Sessenta e um casos de carcinomas uroteliais musculoinvasivos ou localmente avançados primários da bexiga operados no Hospital das Clínicas da Faculdade de Medicina da USP e no Instituto do Câncer do Estado de São Paulo, sendo 34 casos com metástase para linfonodo regional, foram caracterizados quanto ao gênero, idade, tamanho, focalidade, grau histológico, tipo/configuração neoplásica, tipo papilífero da neoplasia, padrão arquitetural de invasão tumoral, grau de atipia nuclear, componente sarcomatoide, diferenciações escamosa e glandular, variante histológica, invasões linfovascular e perineural, carcinoma in situ, estádio do tumor primário, metástase para linfonodo regional, tamanho da metástase e extensão extranodal. Amostras teciduais de 1,0 mm foram dispostas em micromatrizes teciduais (TMA) para pesquisa imuno-histoquímica (IH) das enzimas MMP-2, MMP-9 e MMP-14. A expressão IH das MMPs foi graduada em uma escala semiquantitativa de 0 (ausência de expressão) até 20 (maior expressão). As associações entre a imunoexpressão das MMPs de forma global, no epitélio e estroma do tumor primário e na metástase linfonodal com as variáveis anatomopatológicas foram avaliadas através do teste do qui-quadrado de Pearson, sendo consideradas significativas ao nível de p<0,05. RESULTADOS: Trinta e seis, 57 e 60 casos do tumor primário foram positivos para MMP-2, MMP-9 e MMP-14, respectivamente. Nas metástases linfonodais, 20, 27 e 26 casos foram positivos para MMP-2, MMP-9 e MMP-14, respectivamente. A imunoexpressão global de MMP-2 no tumor primário mostrou-se associada com o padrão arquitetural de invasão (p=0,022) e sua expressão no estroma com o grau de atipia...

OBJECTIVES: To study morphological features related to tumor progression in urothelial carcinoma of the urinary bladder and its association with immunohistochemical (IHC) expression of matrix metalloproteinases (MMPs) -2, -9 and -14 in epithelial and stromal cells of primary tumor and regional lymph node metastases. METHODS: Sixty-one cases of muscle-invasive or locally advanced urothelial carcinomas of the bladder operated on Clinic's Hospital of Faculty Medicine Sao Paulo University and the Cancer Institute of the State of Sao Paulo, with 34 cases showing regional lymph nodes metastases, were characterized regarding gender, age, tumor size, multifocality, histological grade, neoplastic type/configuration, papillary type, architectural pattern of invasive tumor, nuclear atypia, sarcomatoid component, squamous and glandular diffentiation, histological variants, lymphovascular and perineural invasion, carcinoma in situ, tumor stage, metastases to regional lymph nodes, metastases size and extranodal extension. Tissue samples of 1.0 mm were arranged in tissue microarrays blocks (TMA) for IHC detection of MMP-2, MMP-9 and MMP-14. The grading of expression of MMPs was determined to a semiquantitative scale from 0 (absence) to 20 (higher expression). The associations between the IHC global expression of MMPs, in epithelium and in stromal cells of the primary tumor and in the lymph node metastases with the morphological features were obtained through Pearson's chi-square (significant at p<0.05). RESULTS: Thirty-six, 57 and 60 cases of primary tumor were positive for MMP-2, MMP-9 and MMP-14 respectively. In the lymph nodes metastases, 20, 27 and 26 cases were positive for MMP-2, MMP-9 and MMP-14 respectively. The global IHC expression of MMP-2 in primary tumor has been associated with the architectural pattern of invasion (p=0.022). The expression in stromal cells were correlated with the degree of nuclear atypia (p=0.032) and the percentage of sarcomatoid...

Clinically low-risk prostate cancer: evaluation with transrectal doppler ultrasound and functional magnetic resonance imaging

OBJECTIVES: To evaluate transrectal ultrasound, amplitude Doppler ultrasound, conventional T2-weighted magnetic resonance imaging, spectroscopy and dynamic contrast-enhanced magnetic resonance imaging in localizing and locally staging low-risk prostate cancer. INTRODUCTION: Prostate cancer has been diagnosed at earlier stages and the most accepted classification for low-risk prostate cancer is based on clinical stage T1c or T2a, Gleason score <6, and prostate-specific antigen (PSA) <10 ng/ml. METHODS: From 2005 to 2006, magnetic resonance imaging was performed in 42 patients, and transrectal ultrasound in 26 of these patients. Seven patients were excluded from the study. Mean patient age was 64.94 years and mean serum PSA was 6.05 ng/ml. The examinations were analyzed for tumor identification and location in prostate sextants, detection of extracapsular extension, and seminal vesicle invasion, using surgical pathology findings as the gold standard. RESULTS: Sixteen patients (45.7 percent) had pathologically proven organ-confined disease, 11 (31.4 percent) had positive surgical margin, 8 (28.9 percent) had extracapsular extension, and 3 (8.6 percent) presented with extracapsular extension and seminal vesicle invasion. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy values for localizing low-risk prostate cancer were 53.1 percent, 48.3 percent, 63.4 percent, 37.8 percent and 51.3 percent for transrectal ultrasound; 70.4 percent, 36.2 percent, 65.1 percent, 42.0 percent and 57.7 percent for amplitude Doppler ultrasound; 71.5 percent, 58.9 percent, 76.6 percent, 52.4 percent and 67.1 percent for magnetic resonance imaging; 70.4 percent, 58.7 percent, 78.4 percent, 48.2 percent and 66.7 percent for magnetic resonance spectroscopy; 67.2 percent, 65.7 percent, 79.3 percent, 50.6 percent and 66.7 percent for dynamic contrast-enhanced magnetic resonance imaging, respectively. Sensitivity, specificity, PPV, NPV and accuracy values for detecting extracapsular extension were 33.3 percent, 92 percent, 14.3 percent, 97.2 percent and 89.7 percent for transrectal ultrasound and 50.0 percent, 77.6 percent, 13.7 percent, 95.6 percent and 75.7 percent for magnetic resonance imaging, respectively. For detecting seminal vesicle invasion, these values were 66.7 percent, 85.7 percent, 22.2 percent, 97.7 percent and 84.6 percent for transrectal ultrasound and 40.0 percent, 83.1 percent, 15.4 percent, 94.7 percent and 80.0 percent for magnetic resonance imaging. CONCLUSION: Although preliminary, our results suggest that imaging modalities have limited usefulness in localizing and locally staging clinically low-risk prostate cancer.

Evaluation of adrenal tumors by magnetic resonance imaging with histological correlation / Avaliação por ressonância magnética dos tumores de adrenal com correlação histológica

A ressonância magnética é ferramenta importante para a detecção e caracterização dos tumores adrenais. O conhecimento das diferentes apresentações dos tumores primários e secundários à ressonância magnética e sua correlação com dados da histologia são essenciais para o correto raciocínio diagnóstico. Este artigo revisa os aspectos que podem estreitar o diagnóstico diferencial dos tumores adrenais, dando ênfase à correlação histológica daqueles mais comuns.

Magnetic resonance imaging is an important tool for the detection and characterization of adrenal tumors. The knowledge about the different presentations of primary and secondary adrenal tumors at magnetic resonance imaging and their correlation with histological data are essential for the establishment of a correct diagnosis. The present study reviews magnetic resonance imaging aspects which may narrow the differential diagnosis of adrenal tumors, emphasizing the histological correlation of the most frequent ones.

Fatal outcome in bronchus-associated lymphoid tissue lymphoma.

Primary pulmonary lymphoma is rare. The most common histological type is the bronchus-associated lymphoid tissue lymphoma. This type of lymphoma has an indolent course and excellent response to therapy. One-third of all cases are diagnosed incidentally. However, due to the rarity of this disease, little is known about its natural history in terms of dissemination and evolution. Herein, we report the unusual case of a 61-year-old man who refused treatment after being diagnosed with bronchus-associated lymphoid tissue lymphoma and died 2 years later from massive lung infiltration without dissemination to other organs.

Linfoma do tecido linfóide associado ao brônquio com evolução fatal / Fatal outcome in bronchus-associated lymphoid tissue lymphoma

Primary pulmonary lymphoma is rare. The most common histological type is the bronchus-associated lymphoid tissue lymphoma. This type of lymphoma has an indolent course and excellent response to therapy. One-third of all cases are diagnosed incidentally. However, due to the rarity of this disease, little is known about its natural history in terms of dissemination and evolution. Herein, we report the unusual case of a 61-year-old man who refused treatment after being diagnosed with bronchus-associated lymphoid tissue lymphoma and died 2 years later from massive lung infiltration without dissemination to other organs.

Linfomas primários do pulmão são raros. O tipo histológico mais freqüente é o linfoma do tecido linfóide associado ao brônquio. Este tipo de linfoma tem curso indolente e excelente resposta à terapia. Um terço dos casos é descoberto incidentalmente. Devido à raridade desta doença, no entanto, pouco se conhece sobre sua história natural em termos de disseminação e evolução. Neste relato, descrevemos o caso incomum de um homem de 61 anos que recusou o tratamento após diagnóstico de linfoma do tecido linfóide associado ao brônquio e, 2 anos após o diagnóstico, morreu por infiltração pulmonar maciça sem disseminação para outros órgãos.