Primary synovial sarcomas of the heart are aggressive and extremely rare tumors. Only thirteen cases have been reported previously. We report the fourteenth case and review the previous ones. Our patient is a 45 year-old man who presented with congestive heart failure, which was related to a right atrial pedunculated ...

Intravenous leiomyomatosis is a rare smooth muscle tumor. We report two cases of intravenous leiomyomatosis that grew along different routes of the venous system into the inferior vena cava and the right atrium. The different route of extension makes a difference in the ease of excision of tumor masses. Using ...

Angiosarcoma is a rare vascular malignant tumor most commonly seen on the scalp of elderly people. We report here two cases of angiosarcoma of the face in 74- and 75-year-old males. It is very unusual to find the development of an angiosarcoma with a rosacea on the face. To the ...

Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of the liver with an unpredictable malignant potential. Its growth can lead to hepatic failure, extrahepatic metastasis and death. Surgical resection or liver transplantation is the treatment of choice if metastasis is not identified. Several antineoplastic agents have been proposed for ...

Paraganglioma and the variant gangliocytic paraganglioma are rare gastrointestinal tumors. We present the first reported case of an esophageal paraganglioma and a review of the literature. From this review it seems that these tumors can occur at any age and usually present with acute or chronic bleeding with or without ...

Aggressive angiomyxoma (AA) is a rare mesenchymal tumor of the lower pelvis and genital region, characterized by local infiltration and frequent, even multiple recurrences. In the present paper a case of a small-sized AA of the vagina, in a 55-year-old woman is reported. We describe the histological appearance and the ...

OBJECTIVES/HYPOTHESIS: The objectives were to present a case report of a woman with recurrent disease during consecutive pregnancies and to discuss the diagnosis and management of granular cell tracheal tumors, with particular attention to pregnancy and hyperestrogenic states. STUDY DESIGN: Case report and literature review. METHODS: A Medline search and ...

Two cases of craniopharyngioma with intratumoral hemorrhage are reported. A 22-year-old male was admitted with meningitis. Lumbar tapping was performed twice. He subsequently developed reduced visual acuity and field deterioration due to intratumoral hemorrhage from an intra- and suprasellar tumor. He underwent emergency craniotomy and total extirpation of the tumor. ...

A 56-year-old woman presented with an intracranial osteosarcoma at the site of previous radiosurgery, manifesting as sudden onset of headache and left hemiparesis with aphasia. She had a previous history of stereotactic radiosurgery for an intracranial tumor under a diagnosis of falx meningioma. Computed tomography showed intratumoral and peritumoral hemorrhage ...

This case report describes the rare phenomenon of encephalopathy associated with massive carcinoid tumor. Extensive investigation failed to reveal an obvious cause but a presumptive diagnosis of tryptophan deficiency was made and she was commenced on tryptophan dietary supplements. A rapid and complete response resulted. This case report discusses this ...

Primary pleural angiosarcoma is an extremely rare tumor. We report the case of a patient who presented with recurrent massive bilateral hemothoraxes. Although thoracoscopy was performed, biopsy samples of the pleura were inconclusive. The delayed onset of skin metastases led to the diagnosis of angiosarcoma, however the patient died from ...

Neurilemmomas are benign tumors which originate from Schwann cells. They rarely occur in the trachea or bronchus. We encountered two cases of endobronchial neurilemmoma and in this context, reviewed 48 cases previously reported in Japan. Neurilemmomas can occur in all regions of the bronchial tree and they often progress into ...

Cutaneous lymphadenoma is a rare tumor with distinctive histologic features. This entity was originally described as lymphoepithelial tumor by Santa Cruz and Barr in 1987. It was renamed cutaneous lymphadenoma in 1991. To date, at least 31 cases of this entity have been reported. The literature did not contain a ...

Knowledge of vulvar rhabdomyosarcoma (RMS) is based on case reports and collective reviews since the pathology is extremely rare and experience of individual institutions limited. We present a case of a 15-year-old girl with large-sized vulvar embryonal RMS. The patient was treated with wide local excision of the tumor, multiagent ...

Mixed müllerian tumors (MMT) of the uterus have been reported following the use of tamoxifen. Rarely, these tumors lead to an uterine inversion. The surgical approach of a uterine inversion forms a therapeutic challenge. In this article, a case of uterine inversion due to a MMT is reported, together with ...

Pancreatoblastoma, generally regarded as a pediatric malignant tumor, is rarely found in the adult population. Only 13 adults with pancreatoblastoma, ranging in age from 19 to 68 years, have previously been reported in the world literature. A diagnosis of pancreatoblastoma relies on characteristic histologic features, including epithelial differentiation and, more ...

Well-differentiated fetal adenocarcinoma (WDFA) is a rare tumor of the lung, which has gone by many names over the years. The lesion was first described by Kradin et al., in 1982, who called it "pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung)." Since ...

We report a 38-year-old woman with a malignant catecholamine-secreting vagal paraganglioma. In the preceding year she had intermittent severe frontoparietal headaches. While she was receiving radiotherapy (35 Gy in 15 fractions) she developed palpitations, which steadily worsened over the following three years. A repeat CT scan showed no change in ...

We report a case of atrophic dermatofibrosarcoma protuberans, an uncommon clinical variant. We discuss molecular and genetic mechanisms of dermatofibrosarcoma protuberans tumorigenesis, review the literature on atrophic dermatofibrosarcoma protuberans, and pose a molecular mechanism for the atrophic variant's phenotype. We also describe using Mohs micrographic excision with CD34 immunostains on ...

OBJECTIVES: To determine the value of frozen section examination for the management of small incidental testis tumors. METHODS: We reviewed all cases of incidental nonpalpable testis tumors that have been examined in frozen section during the period 1996-2002. For each case, frozen section and definitive histological slides were available and ...

Case report: A 75-year-old baikarian woman was admitted to our hospital for treatment of seizures. From the results of neurological and radiological examination, a left frontal meningioma was suspected and the patient was referred to our department for neurosurgical intervention. At admission, the MRI showed a basilar impression accompanied by ...

Cystosarcoma phyllodes (CSP) tumors of the breast are rare, comprising less than 1 per cent of all mammary neoplasms. CSP tumors have typically been regarded as predominantly benign, unilateral lesions of the female breast. However, sporadic cases of CSP have been reported in males. We present the first case of ...

Neurofibromatosis (NF) type I (NF1) is the most common familial cancer-predisposing syndrome in humans, while type 2 (NF2) accounts for an extremely small percentage of the total cases of NF. Tumors occurring in patients with NF1 are primarily peripheral neurofibromas, while NF2 patients present with central schwannomas. Malignant transformation has ...

A 6-month-old girl presented with respiratory symptoms with a large right-sided mediastinal mass noted on diagnostic imaging. Percutaneous biopsy revealed normal thymic tissue. Steroids were administered with no response. Right thoracotomy and complete thymectomy were performed. The specimen weighed approximately eight to ten times normal weight, and histology and flow ...

Parotid lipomatosis is extremely rare in children. Only 4 cases have previously been reported in the English language medical literature. Surgical excision is frequently complicated by recurrence. We report, a fifth case, on a 5-month-old girl with rapidly progressive parotid lipomatosis. Emphasis is laid on the importance of preserving the ...

Retroperitoneal mucinous cystadenomas are extremely rare tumors found exclusively in women. An additional case of retroperitoneal mucinous cystadenoma histologically confirmed in a 41-year- old woman is reported herein. Computed tomographic (CT) scanning showed a cystic mass, 21 x 16 cm in size, in the right retroperitoneal space. Removal of the ...

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor that occurs among young women and typically presents as bilateral multiple nodules. In the present report, we describe an uncommon case of PEH presented as a single cavitary nodule in a 33-yr-old asymptomatic man. This is the first case of PEH presented ...

The specific c-kit protein (CD117)-positive gastrointestinal stromal tumor (GIST) of the rectum has been sporadically reported in pathology literature, but its radiologic features have never been thoroughly described. We present 2 cases of histologically and immunohistochemically confirmed rectal GISTs. Their clinical and multimodality imaging features are illustrated, and the relevant ...

BACKGROUND: Cellular angiofibroma (CA) of the vulva is a recently described condition, whose clinical and pathological features are poorly known. METHODS: We have encountered two cases of this very unusual tumor. Their clinical and pathological features were analyzed and compared to those reported in the literature. RESULTS: Both patients were ...

The authors report a rare case of a cortical ependymoma in a 10-year-old boy. The patient presented with complex partial seizures and a well-circumscribed, right frontal cortical mass. Routine microscopy showed a glial tumor with diverse histologic features. Immunohistochemistry and electron microscopy were required to establish the definitive diagnosis of ...

Rhabdoid tumors of the central nervous system are uncommon tumors. About 188 cases have been reported in the literature so far. In this report, we describe a case of a rhabdoid tumor of the thalamus in a 35-year-old male patient. Light microscopic and immunohistochemical features are discussed and the relevant ...

Management options for patients with vestibular schwannomas (acoustic neuromas) include observation, tumor resection, stereotactic radiosurgery, and fractionated radiotherapy. In this report the authors review their 15-year experience with radiosurgery and discuss indications and expectations in relation to the different approaches. They conducted a survey of neurosurgeons to determine management preferences ...

Mesenchymal chondrosarcomas are rare malignant tumors of the bone and soft tissue. Extraskeletal mesenchymal chondrosarcomas, especially those that arise in the central nervous system, are even rarer. Most of those described were intracranially located, with only a very few cases having been reported in an intraspinal region. Therapeutic experience with ...

Cystosarcoma phyllodes is a rare breast tumor with variable malignant potential. Metastasis has been reported in a small percentage of cases. We describe the case of a 52-year-old woman who developed a large facial tumor 1 year after she had undergone a mastectomy for a rapidly enlarging breast neoplasm. The ...

This is a case report of an extraskeletal mesenchymal chondrosarcoma (ESMC) that originally occurred in the retroperitoneum of a 24-year-old female and later metastasized to the left proximal humerus. Mesenchymal chondrosarcomas are very rare in comparison to conventional chondrosarcomas and even more so when arising from an extraskeletal location. In ...

Parasellar paragangliomas are rare tumors. As far as we know, only ten cases are described in the literature. Their clinical, pathological, and radiological features and possible origin are discussed in this article and a review of the literature is given. Additionally, we report a new case of a 51-year-old woman ...

Myxomas are benign, slow growing neoplasms derived from mesenchyme. While these tumors most frequently occur in the myocardium, the other sites most commonly affected are the maxilla and mandible. Nevertheless, myxoma is a very uncommon lesion of the midface, particularly in the pediatric population. We present two reports of infant ...

BACKGROUND: Intravenous leiomyomatosis with intracaval and intracardiac extension has been rarely described in surgical, gynecological, and radiological literatures. Complete excision of the tumor is essential for a favorable outcome. Because of the uniqueness of this tumor having an absent or localized attachment site, its removal is feasible when assisted, prior ...

The authors present a case of primary intracranial extraosseous myxoid chondrosarcoma without any attachment to the cranium or the meninges. The clinical and radiological findings of the primary intraparenchymal tumor are described with a review of the literature concerning cranial and intracranial myxoid chondrosarcoma.

OBJECTIVES: To review the clinical and pathological picture of laryngeal paragangliomas and compare laryngeal paragangliomas with the three other types of laryngeal neuroendocrine neoplasms. STUDY DESIGN: The study presents a case review of a patient with a subglottic laryngeal paraganglioma treated at our institution, with a literature review of the ...

The natural history of brown tumor of the skull base, a rare and benign condition, has not been adequately documented, particularly after nonsurgical treatment and over a long follow-up period. The authors report a case of brown tumor of the ethmoidal sinus, documenting its clinical, neuroimaging, and pathological features with ...

Primary choriocarcinoma of the colon is a very rare tumor, with only six reported cases in the world literature, all but one of which was associated with an adjacent adenocarcinoma. This has led to the suggestion that colonic choriocarcinomas may arise from the more typical adenocarcinoma a process of further ...

We report a case of schwannoma (neurilemmoma) predominantly composed of small cells arranged in rosettes around central collagenous cores and discuss the differential diagnosis of this unusual variant. Schwannoma with giant rosettes, previously designated as neuroblastoma-like schwannoma by Goldblum et al., must be differentiated from neuroblastoma, peripheral neuroectodermal tumors, and ...

Perineuriomas (PN) are uncommon, slowly growing, usually benign tumors composed of well-differentiated perineural cells. Two variants are recognized: intraneural perineuriomas and soft tissue perineurioma, which includes a sclerosing subset of tumors. They are usually reported in the adult population. We present three cases of soft tissue perineuriomas in children. One ...

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease has multiple clinical features with variable courses creating several pitfalls in clinical diagnosis. There are number of reported cases mimicking malignant skeletal tumors such as chondrosarcoma. However, no case of CPPD disease with radiographic noncalcified soft tissue mass has been reported in the ...

A benign mesenchymoma is an unencapsulated soft tissue neoplasm composed of 2 or more mature mesenchymal tissues not normally associated with each other, excluding fibrous connective tissue. No single mesenchymal tissue should predominate with respect to the other mesenchymal elements. Ten well-documented examples of intraoral benign mesenchymoma have been reported ...

Malignant tumors presenting as an inguinal hernia are rare. We present the case of a malignant mixed Mullerian tumor (MMMT) of the ovary growing into an inguinal hernia sac. In this case, magnetic resonance imaging was useful in making a diagnosis of an ovarian neoplasm growing into the inguinal canal, ...

Pilomatricomas are relatively rare tumors of ectodermal origin from the outer root sheath cell of the hair follicle. They are usually asymptomatic, solitary, firm or hard, freely mobile, dermal or subcutaneous nodules. The purpose of this article is to present a case that illustrates the diagnostic difficulty encountered by oral ...

Behçet's disease is a chronic inflammatory disease characterized by recurrent oral aphthae and systemic manifestations. Gastrointestinal involvement is rare. We report a case of ileocolitis secondary to Behçet's successfully treated with tumor necrosis factor-alpha antibody (infliximab) therapy. To our knowledge this is the second reported case of Behçet's ileocolitis successfully ...

AIM: To examine histological nature and clinical process of ureteral carcinosarcomas. METHODS: We report on a new case of carcinosarcoma of the ureter. The literature was reviewed and histological and clinical features were elucidated. RESULTS: Fourteen cases of ureteral carcinosarcomas have been reported. The nomenclature used for these tumors either ...