We report on a rare case of synchronous angiomyolipoma (AML) and 2 oncocytomas (OCs) in the same kidney of a 73-year-old woman. During the course of a follow-up for a long-standing staghorn calculus in the left kidney, a 4-cm right suprarenal mass and a small solid nodule adjacent to a ...

Angiomyolipoma (AML) is a rare benign neoplasm that usually arises in the kidneys, but may rarely originate in sites such as the retroperitoneum, liver and bone. It is characterised by an intimate admixture of blood vessels, smooth muscle and fat. This multiphasic composition allows for its recognition on ultrasound and ...

Desmoplastic small round cell tumor (DSRCT) is a rare clinicopathological entity individualized in 1989. Its etiopathogenesis is still unknown, and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. The objective of this work is to report two new cases of DSRCT and to review the literature to clarify ...

Paratesticular rhabdomyoma is a rare benign tumor, which usually presents as a painless mass in the scrotum or groin. We report the first case of a locally invasive paratesticular rhabdomyoma in a 17-year-old male teenager who presented with chronic scrotal pain. We further review the current literature of this rare ...

Posterior fossa ependymoma usually arise in the fourth ventricle. Though extension of this tumor into the cerebellopontine angle and subarachnoid space through the foramina of Luschka or Magendie is well described, a primary extraaxial cerebellopontine angle location of a posterior fossa ependymoma is distinctly uncommon. The authors report imaging in ...

Primary osteosarcoma of the kidney is a very rare neoplasm with a very poor prognosis and unclear histogenesis. Only 20 cases have been reported in the literature. It has a strong tendency to recur locally and spread to distant sites. We present the clinical case of a 79-year-old man affected ...

Odontogenic Calcificant Cystic Tumor (OCCT) is an infrequent injury. It arises from odontogenic epithelial rests present in the maxilla, jaw or gum. Gorlin and col. described the OCCT for first time as an own pathological entity in 1962. Clinically, the OCCT represents 1% of the odontogenic injuries. It is possible ...

Lipoblastomas are rare benign soft tissue tumors that occur primarily in young children. Most lipoblastomas occur in the extremities, trunk, head, and neck. An intrascrotal location is unusual. We describe the case of a 4-year-old boy with an intrascrotal lipoblastoma and discuss the differential diagnosis in reviewing the literature.

SUMMARY: Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm usually seen in young children and infants. Prognosis for AT/RT is poor, with most patients dying within 1 year of presentation. AT/RT most commonly occurs intracranially. Location in the spine, though previously reported, is rare, and imaging ...

Myoepithelial hamartoma is a very rare submucosal tumor of the stomach. Magnus-Alsleben first described 5 cases of this tumor in 1903. More recently (1993) Vandelli et al. published a review of the literature comprising only 33 cases. Histologically, the tumor is characterized by hypertrophic smooth muscle bands surrounding diverse epithelial ...

Soft-tissue chondromas are rare and benign soft-tissue tumors. Over 95% occur in the hands and feet. They are seen in both sexes equally and mainly occur in patients aged 30-60 years. If excised fully, they do not reoccur, and no cases in the literature have reported malignant transformations.

Mesenchymal chondrosarcomas represent less than 10% of all chondrosarcomas. The majority of these tumors arise from the skeleton; only 22% have an extraosseous origin. Of the extraskeletal locations, the meninges, brain, and thigh are the most common. Involvement of the forearm is a rare occurrence. This case report describes an ...

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal precursor cells of the gastrointestinal tract that may differentiate towards the interstitial cells of Cajal, pacemaker cells regulating autonomous motility of G.I. tract. Grading of GIST has been proven to be as difficult as their classification. Two thirds of GISTs are ...

Paragangliomas are rare benign neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Here, we described a new case of a 75-year-old woman with paraganglioma arising in the middle and posterior cranial fossa with extended destruction of the skull base and clivus. She was admitted to our ...

OBJECT: Intracranial intradural chordomas are extremely rare lesions, and only a few cases have been reported in the neurosurgical literature. The authors performed a retrospective analysis of cases treated at their institutions. They present an illustrative surgical scenario and discuss the published literature, pathogenesis, and histopathological features as well as ...

Angiomyolipoma is apparently a part of a family of neoplasms that derive from perivascular epitheloid cells. It is a rare mesenchymal tumor, usually found in the kidney. Extrarenal angiomyolipoma is uncommon and the most common extrarenal site is the liver. Only two cases of adrenal angiomyolipoma are reported in English ...

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.

The ameloblastoma is a bening odontogenic tumor. The treatment planning in young persons is still not clear. We describe a case report of a young boy who was treated in our unit and we review the different approaches for this type of lesions, which nowadays still not clear in this ...

Adrenal oncocytoma is a rare entity, with 20 cases reported in literature. A functional oncocytoma is extremely rare. We present a case of adrenal oncocytoma in a hypertensive male who had elevated catecholamine levels, which improved after adrenalectomy with decrease in daily antihypertensive requirement.

Carney's triad is a rare pathogenic entity which consists of the association in young women of multiple condromatosis in the lung, gastric leiomyosarcoma, and extradrenal paraganglioma; although the presence of three at the same time is not required for its diagnosis. We present the case of a 27-year-old woman who ...

In the English medical literature, 27 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the second case of a primary retroperitoneal mucinous cystadenocarcinoma in a man. The patient was an 83-year-old man, with a large 26 x 20 x 16-cm retroperitoneal cystic mass causing abdominal discomfort and ...

Intramedullary gangliogliomas are indolent tumors of the spine with rare cases of malignant transformation. Conus medullaris gangliogliomas are extremely rare but are usually associated with good clinical outcome after gross total resection. We describe the first reported case of a ganglioglioma of the conus medullaris that progressed to glioblastoma multiforme ...

The term keratoameloblastoma has been used to describe a histologically heterogeneous group of ameloblastoma variants which have in common the formation of keratin by the ameloblastomatous epithelium. The English language literature contains reports of only 12 cases of keratoameloblastoma, of which 4 cases exhibited a papilliferous component. We report a ...

Chondromyxoid fibroma is an uncommon benign cartilaginous tumor that rarely presents in the sino-nasal region as a locally destructive, erosive lesion. Both clinically and histologically, it is a difficult diagnosis and can be confused with malignant processes such as myxoid chondrosarcoma. Histology of the tumor, especially with a small sample, ...

Carcinosarcomas of the ureter are rare biphasic neoplasms, with distinct malignant epithelial and mesenchymal components. To the authors' knowledge there have been only 12 cases reported in the literature. Primary cartilaginous tumors of the ureter are also rare tumors. Thus far there has been a single case report of an ...

A 72-yr-old acromegalic man, who presented with pain in the left femur, was found to have a metastatic osteosarcoma. Only three cases describing the coexistence of acromegaly and osteosarcoma have been reported by the literature. As the patient didn't have other risk factors for osteosarcoma, the hypothesis that accelerated rate ...

BACKGROUND: Cerebellar hemorrhage caused by cerebellar hemangioblastoma is not a frequent case. We report a case of solid, cerebellar hemangioblastoma, diagnosed 4 years after cerebellar hemorrhage. CASE DESCRIPTION: A 69-year-old man presented with dizziness and gait disturbance. He had a 4-year history of evacuation of cerebellar hemorrhage. Gadolinium-enhanced MRI revealed ...

Tufted angiomas (TAs) are benign vascular tumors, primarily occurring on the trunk and extremities of children younger than 5 years. Few cases occurring on the oral mucosa and in adults have been reported. Although they are typically isolated lesions, TA has been associated with port-wine stains. We describe a case ...

This study reports on the cytogenetic features of a novel case of pediatric Burkitt-like lymphoma (BLL), that adds to the three published. Four groups of cytogenetic abnormalities were detected in the present case: (1) imbalances shared by most germinal center (GC) derived B-cell tumors including BLL (+1q, -6q, -8p, +8q24 ...

Pineoblastoma represents a class of primitive neuroectodermal tumors (PNET) with poorly differentiated neuroepithelial cells that are histologically indistinguishable from medulloblastomas. It is a rare tumor, typically arising in childhood, and to date only a few cytogenetic cases have been published. We report four new cases in which conventional cytogenetics demonstrated ...

Treatment options for primary and secondary liver tumors that cannot be resected or ablated are based on transarterial techniques. Although the majority of these are based on bland and chemoembolization techniques, yttrium-90 microspheres represent an alternate transarterial option. Although the amount of literature on (90)Y does not rival that of ...

Solid pseudopapillary neoplasms of the pancreas (SPNP) are rare pancreatic tumors that occur predominantly in young women, with very few cases reported in men. While the origin of the tumor may be unclear, it is characterized by a distinct histological appearance and a clinical course highlighting its low malignant potential. ...

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that often occurs during the first year of life. More than 90% of MNTIs occur in the head and neck region, with most on the anterior part of the maxillary ridge. MNTI has also been reported in the mediastinum, thigh, ...

We report a case of gastrointestinal stromal tumor (GIST) of the prostate in a 75-year-old man with dysuria and urinary retention. Digital rectal examination revealed a markedly enlarged prostate. The serum level of prostate-specific antigen was 0.2 ng/dL. Imaging studies showed an expanded prostate, measuring 6.7 x 5.6 x 5.5 ...

Gliomas with cartilaginous metaplasia are extremely uncommon and thought to be due to metaplasia of the mesenchymal supportive elements. The tumors are located in the midline, mostly fourth ventricle and rarely in the pons. The present paper describes an unusual case of anaplastic ependymoma with cartilaginous and osseous metaplasia in ...

CASE REPORT: The case of a 6-year-old girl with a pleomorphic xanthoastrocytoma (PXA) in the left cerebellopontine angle (CPA) is reported. The clinical, radiological, and histopathological findings are presented. DISCUSSION: To our knowledge, the presence of PXA in the CPA has not previously been reported. The rare infratentorial locations of ...

BACKGROUND: As conservative treatments for retinoblastoma become more prevalent, there will be a greater number of ocular complications developing in eyes that previously would have been enucleated. Pars plana vitrectomy (PPV) is needed to manage some of these complications. METHODS: The authors evaluated three consecutive cases of PPV in eyes ...

Liposarcomas are rare neoplasms in domestic animals, but have been reported to occur in many species. In humans, liposarcoma is one of the most common malignant mesenchymal tumors. Classification of liposarcomas in humans has been well established and categorization by type can be of prognostic value; no such unique classification ...

Central neurocytomas (CN) are uncommon tumors of the central nervous system, most descriptions of which available in the literature are in the form of isolated case reports and small series. Owing to this rare incidence, diagnosis and management of this neoplasm remain controversial. Usually, these tumors affect lateral ventricles of ...

Lipoblastoma is a rare benign tumor that occurs in infancy and early childhood, accounting to less than 1% of all childhood neoplasms. About 20 cases of mediastinal lipoblastoma have been reported in English literature. We report a rare case of mediastinal lipoblastoma in a 6-year-old boy. The tumor weighing 996 ...

A rare case of hemangioblastoma located in the region of hippocampus is reported. A 27-year-old female presented with a single episode of generalized convulsion. The vascular and cherry red color hemangioblastoma was resected by a temporo-zygomatic approach. There has been no recurrence of tumor at a follow-up of 11 years

BACKGROUND: Malignant fibrous histiocytoma (MFH) in the large bowel, which is composed of spindle-shaped cells arranged in a pleiomorphic and storiform pattern, is an extremely rare tumor. METHODS: We in this study report on a case of a 73-year-old man with a sarcoma arising from a diverticular sigmoid without any ...

BACKGROUND: Organs of Zuckerkandl (O of Z) harbor the potential for deadly paragangliomas. Paragangliomas are one of the surgical causes of hypertension. Major treatises of medicine offer very little information on this topic. METHODS: PubMed Medline and Google searches were performed to obtain reported cases of paragangliomas of the O ...

Although it is generally recognized that some benign sweat gland neoplasms may show appreciable mitotic activity, there are few reports of its quantitative analysis in specific tumor types or of its correlation with clinical behavior. The presence of a large number of mitoses in a sweat gland tumor for which ...

BACKGROUND: We report an additional case of an extremely uncommon but distinctive histological variant of benign schwannoma, which was previously designated as neuroblastoma-like schwannoma by Goldblum et al. METHODS: A 29-year-old woman referred to a 6-year-history of an atrofic macule. Its clinical appearance was similar to that of an anetoderma. ...

Bronchopulmonary carcinoid tumors have been associated with a variety of endocrine disorders including Cushing's syndrome (CS), which is caused by ectopic adrenocorticotrophic hormone (ACTH) secretion. We report two cases of CS secondary to bronchopulmonary carcinoid tumors. The first patient, a 29-year-old woman, presented hypokalemia, high serum ACTH level and high ...

Hirsutism is a symptom or sign, which may have more serious associations than cosmetic and psychological concern alone, such as adrenal hyperplasia and ovarian tumor, particularly if it develops well after puberty. Some medicines having androgenic activity may also cause this problem. Here, we present a case of a young ...

OBJECTIVE: Pure pineal germinomas have been rarely reported in girls. Gender incidence and differences of pure pineal germinomas are not well known. The authors report a series of pure pineal germinoma and its gender characteristic is reviewed. METHODS AND RESULTS: Of a total of 50 germ cell tumors operated on ...

Two brothers with familial carcinoid tumors of the rectum are presented. A few cases documenting the occurrence of carcinoid tumors in first-degree relatives in the absence of the multiple endocrine neoplasia (MEN) syndromes have been reported in the literature. Consistent with these previous reports, in this case both patients had ...

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, ...