Intraabdominal desmoplastic small round cell tumor: Report of a case and literature review.

INTRODUCTION: Desmoplastic small round cell tumor is a rare malignancy with poor prognosis that predominantly affects young males. Its etiopathogenesis is still unknown and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis. CASE PRESENTATION: We report a new case of a 29-year-old male who proceeded to our Emergency Department complaining about non-specific abdominal pain. Physical examination revealed no abnormalities except for a palpable mass in the lower abdomen and a diffuse abdominal pain. Computed Tomography scan showed enlarged paraortic and mesenteric lymphadenopathy, thickness of the small bowel wall and dispersed masses intraperitoneally. He underwent an exploratory laparotomy and the resultant biopsy revealed desmoplastic small round cell tumor. DISCUSSION: Diagnosis of desmoplastic small round cell tumor can easily be missed because it presents with few early warning symptoms and signs, while the routine blood tests are within normal limits. CONCLUSION: A high degree of suspicion, a thorough physical examination, a full imaging check and an aggressive therapeutic approach are required in order to identify this disease and fight for a better quality of life for these patients. In addition we make a review of the literature in an effort to clarify the epidemiological, clinical and pathological aspects of this entity.

A nonphosphaturic mesenchymal tumor mixed connective tissue variant of the sacrum.

Tumor-induced or oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by overproduction of fibroblast growth factor-23 as a phosphaturic agent and renal phosphate wasting. A range of predominantly mesenchymal neoplasms have been associated with tumor-induced osteomalacia and classified as phosphaturic mesenchymal tumor mixed connective tissues. However, phosphaturic mesenchymal tumor mixed connective tissues could be nonphosphaturic in the first stage of the disease, either because the tumors are resected early in the clinical course or because the patient's osteomalacia was attributed to another cause. This article presents a case of a 42-year-old woman with a 2-year history of low back and right leg pain. Laboratory examinations including serum and urine calcium and phosphorous were within normal values. Imaging of the lumbar spine and pelvis showed an osteolytic lesion occupying the right sacral wing. Histology was unclear. Reverse-transcription polymerase chain reaction analysis for fibroblast growth factor-23 was positive and confirmed the diagnosis of phosphaturic mesenchymal tumor mixed connective tissues. Preoperative selective arterial embolization and complete intralesional excision, bone grafting, and instrumented fusion from L4 to L5 to the iliac wings bilaterally was performed. Postoperative recovery was uneventful. Neurological deficits were not observed. A lumbopelvic corset was applied for 3 months. At 12 months, the patient was asymptomatic. Serum and urine values of calcium and phosphorous were normal throughout the follow-up evaluation.

Sacral epidural noncommunicating arachnoid cyst. Case report and review of the literature.

The authors examine the natural history of a spinal epidural arachnoid cyst and present their experience with its treatment in a 25-year-old man who presented with progressive cauda equina syndrome. Neuroimaging revealed two neighboring sacral epidural cysts. The cysts were completely removed via a sacral S1-4 laminectomy; no communication with the subarachnoid space could be found. The patient's postoperative course was uneventful. He experienced progressive improvement and, finally, complete resolution of symptoms and no recurrence of the cyst. Nabors Type I sacral epidural arachnoid cysts are rare; in some cases their origins and the mechanism by which they cause deterioration in the patients' clinical condition are debatable. Findings in the present case support the idea that some of these cysts are noncommunicating but progressive in their clinical presentation. This lesion type is also known to occur intracranially. A brief review of the literature is provided.

Hemangiopericytoma-like synovial sarcoma of the lumbar spine. Case report.

The purpose of this report is to demonstrate that synovial sarcoma should be included in the differential diagnosis of tumors originating from the lumbar spine, especially if they show hemangiopericytoma-like pathological characteristics. A synovial sarcoma is a mesenchymal spindle cell tumor that displays variable epithelial differentiation including glandular formation. It is unrelated to a synovium. More than 80% of these lesions arise in the deep soft tissue of the extremities. The tumor frequently arises adjacent to joints or tendon sheaths. The authors describe a young woman with a hemangiopericytoma-like tumor of the lumbar spine. During repeated operation, this lesion was shown to be a synovial sarcoma, which had invaded the dura mater. The tumor metastasized to the mediastinum and the intradural cervical spine and, finally, to the brain and the lungs. To the authors' knowledge, this is the first reported case of a synovial sarcoma originating from the lumbar spine.

Osteoid osteoma of the patella: a case report.

Though osteoid osteoma is a common primary benign lesion of the bones, intra-articular involvement is rare and poses diagnostic difficulties when it affects middle-aged patients. We present the case of a 51-year-old woman with a 2.5 year history of anterior knee pain that was misdiagnosed as osteochondritis dissecans. Radiological findings were absent, whereas MRI showed a well-circumscribed lesion. A local excisional biopsy was performed and microscopic appearance confirmed diagnosis.

Glomus tumor: a rare location in the upper lip.

Glomus tumor is a benign neoplasm composed of glomocytes and vessels in varying proportions. It appears as a single painful nodule typically found in the dermis and the subcutaneous tissue of the extremities. A rare appearance of the tumor in the upper lip is presented. Differential diagnosis (along with clinical, microscopic, and immunohistochemical findings) is discussed.

Echinococcal synovitis of the knee joint.

Bone lesions are present in 1% to 2% of cases of hydatid disease. Hydatid synovitis can usually be identified due to secondary extension from the adjacent bone, or infrequently after hematogenous spread. We present an extremely rare case of hydatid synovitis without bony involvement. A 74-year-old man with diagnosed hydatid disease was admitted to our department because of left knee swelling. Neither physical examination nor laboratory studies revealed any remarkable findings. Radiographic evaluation of the knee joint was noncontributory. The patient underwent an arthroscopically assisted synovectomy, and the biopsy revealed an echinococcus contamination. No complications occurred during the postoperative period.