The 2004 World Health Organization (WHO) classification of kidney tumors recognizes multilocular cystic renal cell carcinoma (MCRCC) as a rare variant of clear cell renal cell carcinoma with a good prognosis. Available information on its clinical significance is limited. The study cohort included 45 MCRCC cases classified according to 2004 WHO criteria obtained through a multi-institutional international search. Most patients had unilateral MCRCC with no side predominance that was found incidentally; 62% were men, but women had tumors at an earlier age (P = .385). MCRCC occurred slightly more often in men than in women (1.7:1). At diagnosis, 82% of patients had stage T1 and 16%, stage T2; 1 patient had stage T3. The Fuhrman grade was 1 (62%) or 2 (38%), with smaller tumors (

The 2004 World Health Organization (WHO) classification of kidney tumors recognizes multilocular cystic renal cell carcinoma (MCRCC) as a rare variant of clear cell renal cell carcinoma with a good prognosis. Available information on its clinical significance is limited. The study cohort included 45 MCRCC cases classified according to 2004 WHO criteria obtained through a multi-institutional international search. Most patients had unilateral MCRCC with no side predominance that was found incidentally; 62% were men, but women had tumors at an earlier age (P = .385). MCRCC occurred slightly more often in men than in women (1.7:1). At diagnosis, 82% of patients had stage T1 and 16%, stage T2; 1 patient had stage T3. The Fuhrman grade was 1 (62%) or 2 (38%), with smaller tumors (

Financial support: none suMMary Primary osteosarcoma of the breast (POB) is an extremely rare and aggressive tumor. Differential diagnosis of POB includes osteosarcoma of the chest wall and metaplastic breast carcinoma. Imaging tests that exclude the existence of a direct connection between the tumor and chest wall, as well as histopathological and immunohistochemical studies that rule out the presence of an epithelial component are required for the diagnosis of POB. We report a case of a 69-year old woman with POB. Imaging and pathological findings are presented. Therapeutic approach is discussed in the light of current knowledge, including potential complications.Keywords: breast, breast neoplasms, sarcoma, immunochemistry.A 69-year-old woman presented with a 6-month history of a palpable, painless mass in her left breast. Clinical examination revealed a hard, mobile, well-circumscribed, 10 cm mass, occupying practically the entire left breast. Contralateral breast, axilla or nipples showed no abnormalities. She denied having any history of breast trauma, radiotherapy, or breast cancer.Mammography revealed a relatively well-defined, hyperdense irregular mass without calcifications, unconnected to the underlying sternum and ribs ( Figure 1A). Ultrasound revealed a hypoechoic irregular mass with hyperechoic areas, indistinct margins, and posterior acoustic shadowing ( Figure 1B). Core needle biopsy was performed and microscopy showed a malignant poorly differentiated mesenchymal tumor.Thoracic and abdominal computed tomography showed no distant metastasis. The patient underwent simple mastectomy and sentinel lymph node biopsy. Grossly, the cut surface of the tumor was white, hard, with a stony consistency in the center and well-defined margins. The tumor measured 10x9x7 cm (Figure 2).Microscopy showed a malignant immature mesenchymal bone-forming tumor. There were foci of necrosis and cartilage, containing osteoid matrix, some of which were irregularly calcified, surrounded by atypical spindle cells and osteoclast-like multinucleated giant cells. Tumor mitotic count was 16 mitoses/10 high-power field. There was no skin and lymphovascular invasion. Sentinel lymph node was tumor-free. On immunohistochemistry, tumor cells were positive for vimentin. CD68 (KP1) and alpha-1-antitrypsin were positive in osteoclast-like giant cells. Cytokeratin (AE1/AE3), S-100, epithelial membrane antigen (EMA), p63, epidermal growth factor receptor (EGFR), CD99, estrogen and progesterone receptors, and HER2 were negative. The Ki-67 index was 5%. Extensive tumor sampling failed to identify any tumor that was biphasic or had epithelial component. A highgrade primary osteosarcoma of the breast (POB) was di-

Introduction: Fetal congenital cystic adenomatoid malformation is a pulmonary developmental anomaly arising from an overgrowth of the terminal respiratory bronchioles. This is such a rare malformation that it is not always thought of as a diagnostic possibility. Case: In the present case, after a large pulmonary cyst started deviating the mediastinum and following an increase in amniotic fluid volume in the fetus at 28 weeks of gestation, the placement of a catheter for continuous drainage was performed. Subsequent follow-up with ultrasounds and serial echocardiograms revealed normalization of the position of mediastinum, normal amniotic fluid levels, and the correct position of the catheter. The size of the cyst was decreasing daily, but 8 weeks after fetal invasive procedure, there was concern about the stabilization of its size, with the suspicion of obstruction of the catheter. Cesarean delivery was performed at 38 weeks’ gestation. The size of the cyst on the day of C-section was 3.0 × 2.9 × 2.1 cm. At delivery, the infant weighed 3,030 g, with no evidence of respiratory distress, revealing the correct location of catheter. At day 4 after delivery a superior right pulmonary lobe resection was performed. Conclusion: Large pulmonary cysts may produce deviation of mediastinum and can lead to cardiac tamponade, nonimmune hydrops and pulmonary hypoplasia. Precise prenatal imaging and different fetal therapeutic strategies may allow survival of affected fetuses.

Rev Bras Ginecol Obstet. 2005; 27(6): Relato de Caso RESUMO A malformação adenomatóide cística fetal consiste em anomalia do desenvolvimento pulmonar, proveniente de crescimento anormal dos bronquíolos respiratórios terminais. Por tratar-se de malformação rara, nem sempre é lembrada como possibilidade diagnóstica. Apresentamos o relato de um caso de malformação adenomatóide cística pulmonar fetal em adolescente primigesta, ressaltando a importância do seu diagnóstico precoce e possibilidades terapêuticas. Apresentamos também o aspecto evidenciado após a colocação de cateter para drenagem contínua. PALAVRAS-CHAVE: Diagnóstico pré-natal; Feto/anormalidades; Malformação adenomatóide cística congênita do pulmãoABSTRACT Fetal cystic adenomatoid malformation is a pulmonary developmental anomaly arising from an overgrowth of the terminal respiratory bronchioles. This is such a rare malformation, that is not always thought of as a diagnostic possibility. We present a case of pulmonary cystic adenomatoid malformation and emphasize the importance of early diagnosis and therapeutic possibilities. We also present its evolution after prenatal placement of a catheter for continuous drainage. IntroduçãoAs alterações císticas envolvendo as estruturas intratorácicas fetais são, em sua maioria, acessíveis à propedêutica ultra-sonográfica durante o período intra-uterino.Na avaliação do tórax fetal normal, os órgãos identificáveis são os pulmões e o coração, entretanto, em face de situações patológicas, estruturas torácicas invisíveis ao exame normal, tais como, espaço pleural, espaço pericárdico e mediastino, são de fundamental importância para se firmar um diagnóstico provável e conduzir apropriadamente cada situação 1 .A malformação adenomatóide cística pulmonar (MACP) é caracterizada por anomalia de desenvolvimento do epitélio bronquiolar endodérmico e seu mesênquima adjacente originando a proliferação excessiva das estruturas respiratórias terminais. Esta falha embrionária ocorre entre a 6ª e a 8ª semana após a concepção, sendo que algumas lesões podem se desenvolver mais tardiamente entre a 14ª e a 18ª semana da gravidez 2 .Diagnóstico e conduta pré-natal em malformação adenomatóide cística pulmonar fetal: apresentação de um caso Prenatal diagnosis and therapy for fetal cystic adenomatoid pulmonary