Inflammatory myofibroblastic tumor of the heart

An inflammatory myofibroblastic tumor (IMFT) is recognized as benign tissue proliferative response comprising a variety of inflammatory and mesenchymal cells, and presents commonly at a young age. Although it occurs most frequently in the lung, it has also been observed in other organs and tissues such as the liver, spleen, bladder, and lymph nodes. However, IMFT of the heart is rare, and previously only 38 cases have been reported in the English literature. We herein report the case of a 65-year-old woman with asymptomatic IMFT in the right ventricular outflow tract. Previously reported cases are reviewed.     

Hepatocellular carcinoma: a case revealed by metastatic orbital tumor

INTRODUCTION: We report a case of metastatic orbital tumor revealing hepatocellular carcinoma. EXEGESIS: Metastatic orbital tumors of hepatocarcinoma are rare. Only six cases have been reported. We compare these cases to our observation. Treatment of the orbital metastasis is important to decrease pain, ophthalmological symptoms and to improve the quality of survival. Radiotherapy and/or surgery can be used. Prognosis for life depends on liver involvement: the modalities of treatment of the hepatocarcinoma have to be discussed for each patient. CONCLUSION: Seven cases of orbital metastasis revealing a hepatocarcinoma have been documented. Effectiveness of radiotherapy makes the local prognosis good, but prognosis depends on liver involvement, since prognosis of hepatocellular carcinoma is poor.     

Sclerosing Sertoli cell tumor of the testis

Sclerosing Sertoli cell tumor of the testis is a very rare neoplasm. To date only 19 cases have been reported in the literature. We present the twentieth case in a 39 yearold man treated with conservative surgery with a review of the pathological and urological literature.     

Amylase: sensitive tumor marker for amylase-producing lung adenocarcinoma

Hyperamylasemia in patients with lung cancer is rarely, comprising 1% to 3% of all lung cancers. This report describes two cases of lung adenocarcinoma coexisting with hyperamylasemia in two women aged 77 and 57, respectively. In these two cases, CT revealed a normal pancreas. We monitored the serum and urine amylase levels during therapy and found it paralleled tumor response to chemotherapy and metastasis. We suggest that the amylase levels are related to the tumor size and might be a valuable factor in predicting chemotherapy and progression of disease for amylase-producing lung cancer.KEY WORDS : Hyperamylasemia, lung adenocarcinoma, chemotherapy     

Glomus tumor in the lung parenchyma

Glomus tumors are uncommon lesions of glomus cell origin with ultrastructural and immunohistochemical features of smooth muscle. In the majority of the cases reported in the literature glomus tumors are benign, but there are some rare cases in which they demonstrate aggressive and malignant clinical and histological features. Glomangiosarcomas of the lung are extremely rare malignant tumors, because of the fact that glomus bodies are rare or absent. Due to their rarity they are misdiagnosed. Surgical resection is considered to be the most effective treatment for this condition. We report a case of a 35 year old female with a pulmonary glomangiosarcoma.KEY WORDS : Glomangiosarcoma, misdiagnosis, surgical resection     

Thymic epithelial tumor with superior vena cava syndrome diagnosed by percutaneous endovascular tumor biopsy: A report of two cases

Thymic epithelial tumor is a rare, potentially progressive disease that commonly infiltrates mediastinal structures. In rare cases, it may cause superior vena cava syndrome. Pretreatment histopathological diagnosis is essential to determine the most effective treatment strategy. Percutaneous endovascular biopsy is a rarely reported non-surgical diagnostic option for large vessel tumoral involvement. We report two cases of thymic epithelial tumor with superior vena cava syndrome diagnosed by percutaneous endovascular biopsy. No procedural complications occurred, and subsequent systemic treatment was promptly administered. This procedure may have potential as a useful diagnostic method for patients with mediastinal tumors involving large vessels.     

Extrinsic impression or submucous tumor? Contribution of endosonography to clinical assessment]

Impressions of the lumen are frequently found during EGD. We examined 47 patients with 51 impressions by endoscopic ultrasonography and could clear their origin in 44 cases. We found 8 submucosal tumors, 1 submucosal cyst, 5 cysts and 2 malignancies of organs in the neighbourhood; in 28 cases the impression was caused by anatomical variations. In our opinion endoscopic ultrasonography is the method of choice in clearing the nature of impressions of the upper GI-tract.     

Conservative management of pituitary tumor apoplexy

Pituitary tumor apoplexy is a rare neuroendocrine syndrome resulting, in most cases, from hemorrhage or infarctation of a pre-existing pituitary adenoma. Treatment recommendations vary; some authors advocate urgent surgical decompression of the tumor, whereas others suggest that conservative management can lead to recovery of neuro-ophthalmologic function. We describe two patients with pituitary tumor apoplexy who had clinically non-functioning macroadenomas and hypopituitarism, including hypogonadism. They were treated conservatively without surgery, and achieved tumor remission.     

The figure-L unilateral mini-sternotomy for anterior mediastinal tumor

We performed a figure-L unilateral mini-sternotomy for anterior mediastinal tumor resection in ten patients. Pathological diagnosis among the ten included six thymomas, three mature teratomas including one with a seminoma component, and one thymic cyst. Mean tumor length was 7 x 5 x 4 cm. The required skin incision was 8 cm in mean length. The third intercostal space was transected in six cases, the fourth intercostal space in four cases. Bilateral internal thoracic arteries were preserved in all cases. All tumors were completely resected without complications during the procedure. Mean operating time was 106 minutes (range 85 to 120 minutes). Postoperative hospital stay ranged from three to seven days without any complications. All patients were alive at the end of a mean follow-up period of 39 months (range 3 to 60 months). The figure-L unilateral mini-sternotomy is considered an effective and useful minimally invasive approach for anterior mediastinal tumors.     

Pneumocephalus and cerebrospinal fluid fistula following removal of a superior sulcus tumor (pancoast tumor)

Pneumocephalus resulting from a subarachnoid-pleural fistula following resection of a pulmonary neoplasm is a very rare postoperative complication: we have found only 17 cases in the literature. We report the case of a 65-year-old man diagnosed with a superior sulcus tumor who underwent a left upper lobectomy and costal resection following neoadjuvant radiation therapy. Soon after surgery, the patient developed a subarachnoid-pleural fistula and pneumocephalus that required reoperation and drainage. Recurrence was suspected when chronic aseptic meningitis developed. Another thoracotomy was required to resolve the complication.     

Solitary parietal lobe histoplasmoma mimicking a brain tumor

We describe a rare case of Histoplasma infection, which manifested only as a brain histoplasmoma, in a previously healthy woman who had no underlying predisposing conditions. Only a few cases of such intracranial histoplasmomas have been reported but this entity can mimic a brain neoplasm and should be a diagnostic consideration during the evaluation of ring-enhancing brain lesions.     

Non-seminomatous mediastinal germ cell tumor and acute megakaryoblastic leukemia

The association between mediastinal germ cell tumors (MGCT) and acute megakaryoblastic (M7) leukemia has been known for many years. We hereby present this review to better characterize the coexistence of these entities as well as the salient features, the treatment options, and the overall prognosis. A search of PUBMED, Medline, and EMBASE databases via OVID engine for primary articles and case reports under keywords “germ cell tumors” and “acute myeloid leukemia” revealed a total of 26 cases in English that reported MGCT and M7 leukemia. The median age at diagnosis of MGCT was 24 (13–36) years. All cases were stage III. All cases of MGCT were of non-seminomatous origin and one case was unclassified. MGCT occurred prior to the diagnosis of leukemia in 46% of cases and concomitantly in 31% of cases. M7 leukemia was never reported prior to the appearance of MGCT. Complex cytogenetics and hyperdiploidy were the most commonly reported cytogenetic abnormalities. In the 23 cases where the treatment regimen was available, platinum-based chemotherapy directed towards management of the germ cell tumors was used initially in 21 cases and leukemia-directed treatment was used initially in 2 cases only. The median time from diagnosis of MGCT to development of M7 leukemia was 5 (2.25–39) months. Median time to death from the initial diagnosis of MGCT was 6 (0.5–60) months. Patients with a history of MGCT are at higher risk of developing M7 leukemia. They need long-term follow-up with a particular attention to the development of hematological malignancies. The overall prognosis remains poor.     

Primary liposarcoma of the liver--a rare mesenchymal tumor

Primary sarcoms of the liver are rare. Most often angiosarcomas have been reported. Primary liposarcoma of the liver is extremely rare. We report a case of primary liposarcoma of the liver in a 48-year-old woman and compare it with the 13 cases found in the literature. Histologic analysis showed a well differentiated liposarcoma. The treatment of choice is wide local tumor resection.     

Unusual case of pentastomiasis mimicking liver tumor

Pentastomiasis is a rare zoonotic disease. Almost all recorded cases of human pentastomiasis had been incidental findings at autopsy. We report an unusual case of human pentastomiasis mimicking liver tumor successfully treated by liver resection. This clinical presentation is uncommon and it was probably caused by a pentastomid that exited its cyst and migrated to the liver causing an infarct that was mistaken as a primary liver tumor. Diagnosis could not be made before the surgery. This is the first reported case of human pentastomiasis in Brazil.     

口腔颌面部恶性肿瘤肺转移的临床分析——附24例临床病例报道

目的探讨口腔颌面部恶性肿瘤肺转移的诊断及治疗方法。方法从我科2003年1月至2008年12月期间收治的220例口腔颌面部恶性肿瘤患者的临床资料中选取发生肺转移的案例24例,分析肺转移类型,相应的诊疗方法和临床疗效。结果24例肺转移患者中,孤立肺转移10例,双肺多发转移14例;4例患者放弃治疗,20例行全身化疗;所有患者随访6—36个月,其中6～12个月死亡4例,12～24个月死亡16例,4例生存超过24个月。结论早期肺转移患者多无明显呼吸道症状,故x线胸片及胸部CT检查是主要的诊断手段。对于局限性肺转移者可选择X线刀、适形放疗及全身化疗,对于弥散性肺转移者全身化疗是主要治疗手段。     

Granular cell tumor of the esophagus: description of an infrequent benign tumor

Granular cell tumors (GCT) are infrequent tumors first described by Abrikossoff in 1926. Gastrointestinal involvement occurs in about 6% of GCT, the esophagus being the most frequent location. These tumors are usually benign and asymptomatic and are usually found incidentally when an upper gastrointestinal endoscopy is carried out for another reason, showing an isolated polyp or sessile submucosal nodule, covered by intact yellowish mucosa and with firm consistency. Endoscopic ultrasonography has significantly improved the diagnosis of these lesions. Nowadays endoscopic mucosectomy is the treatment of choice of esophageal GCT with a low frequency of complications. Histologic analysis of the surgical specimen shows specific characteristics such as positivity for S-100 protein. We present two new cases of esophageal GCT that were diagnosed recently and discuss the most relevant features of this infrequent disease.     

误诊为肿瘤的腹腔结核

目的 探讨腹腔结核（TB)的临床病理特征和诊断。方法 对1962－1997年间，临床上被误诊为肿瘤（其中31例被误诊为恶性肿瘤），术后经病理检查确诊的45例腹腔TB作回顾性研究。结果 腹腔TB以青年人（≤40岁者29例，64％）和女性（28例，62％）多见。临床主要表现为腹部包块37例（82％）和腹痛30例（67％），其中伴发热17例（38％）、腹胀、便秘或腹泻15例（33％）、体重减轻14例（29％）。手术所见：病变均为肠、腹膜和淋巴结多处受累，病变处有大小不等的包块和结节，酷似癌。镜检：增生型25例，干酪样坏死型14例，混合型6例。结论 腹腔TB的临床诊断与癌的鉴别困难。要提高对腹腔TB临床病理特征的认识，对经各种检查难以确诊的病例，作剖腹探查活检是必要的，以免造成误诊误治。     

Palliative laser therapy of colorectal cancer with sustained local tumor removal

Laser photocoagulation is an established palliative therapeutic method in advanced colorectal cancer. In most cases tumor obstruction of the colonic lumen and symptoms like bleeding, secretion and diarrhoea can be improved or prevented. Even curative tumor therapy of some patients has been reported, particularly in cases with superficial growth of the tumor. We report about an elderly female patient with locally advanced invasive carcinoma of the rectosigmoidal junction. This tumor and a polypoid carcinoma of the sigmoid, possibly in combination with snare resection, were persistently removed by laser photocoagulation.     

Endocrine tumor arising in heterotopic gastric pancreas

We report the case of a 49-year-old caucasian woman, in whom an endocrine tumor arising in gastric heterotopic pancreas was diagnosed. The patient was treated surgically with a gastric wedge resection. Heterotopic pancreas is a benign anatomic condition, probably widely underdiagnosed because usually asymptomatic. The malignant transformation of aberrant pancreas is very rare and almost always in adenocarcinoma. The endocrine tumors developed in heterotopic pancreas are exceedingly rare. Of our knowledge, only four cases have been published and only one case in the gastric location similar to this reported case.     

Tuberous sclerosis and cardiac tumor (case report)

We report the case of a cardiac tumor, which is found in a systematic cardiac examination of an 11 year old girl who had a tuberous sclerosis. The interest of this observation is to show the importance of a general examination, especially cardiac, within this pathology. Tuberous sclerosis is an hereditary disease, associated with rhabdomyomas in 50% of cases or more. Cardiac rhabdomyomas are frequently multiple and detected in utero in some cases. Conversely, 60% of these tumors are seen in a context of tuberous sclerosis. Rhabdomyomas are the most common cardiac tumors of infants and children, the large majority occurring in patients younger than one year and are diagnosed more and more in foetal echocardiography.