Idiopathic massive osteolysis of skull bones: a case report

A rare case of idiopathic massive osteolysis involving cranial bone is reported. Radiological and computed tomographic findings are discussed, along with a brief review of the literature.     

Idiopathic multicentric osteolysis with acro-osteolysis. A case report

We report a case of multicentric massive osteolysis. A 52-year-old woman presented with a three-year history of progressive deformities of the hands. She had osteolytic lesions of the metacarpals and metatarsals, and resorption of the terminal phalanges. During follow-up over four years osteolysis spread to affect the ribs, clavicles, mandible, and long bones. There was no family history of any bone disorder and renal function was normal. Death resulted from resorption of the rib cage and post-mortem studies failed to reveal the cause of the osteolysis.     

Post-fracture osteolysis of the pubic bone simulating a malignancy: report of a case

Post-fracture osteolysis of the pubic bone is rare. We report a case of a 70-year-old woman with osteoporosis and a history of radiation therapy 2 years earlier. At presentation, she was found to have a bilateral sacral fracture and fractures of both pubic rami on one side. The pain persisted, and follow-up radiographs showed osteolysis of the pubic rami suggestive of metastatic disease. The development of a bony callus within 8 months established the diagnosis of benign osteolysis. About 50 cases of osteolysis at fracture sites have been reported to date, of which about a dozen occurred after radiation therapy. All the patients were elderly women with post-menopausal osteoporosis. Radiation therapy probably further increases the risk in this setting. The possibility of osteolysis at fracture sites in patients with osteoporosis should be borne in mind to avoid unnecessary and burdensome investigations that are costly and cause undue anxiety to the patients. Rest is the only effective treatment.     

Histiocytic osteolysis secondary to hyperbilirubinaemia: a case report

A 6-year-old boy with Alagille syndrome, characterised by marked hyperbilirubinaemia, presented with malunion of a pathological fracture of the femur with local bone atrophy and insufficient callus formation. During corrective osteotomy, it was noted that the femur was stained dark green, suggestive of bilirubin deposition. Histology of the resected bone revealed the presence of many histiocytes and osteoclast-like multinucleate giant cells containing bilirubin particles in the cytoplasm causing bone resorption. These findings suggest that bilirubin may activate macrophages to form osteoclast-like multinucleate giant cells, resulting in histiocytic osteolysis.     

Idiopathic phalangeal acroosteolysis: a case report

Acroosteolysis is characterized by bone resorption in the fingers and toes and can occur in several diseases. Phalangeal acroosteolysis is a rare idiopathic form. We report a case in a 13-year-old girl with a 2-year history of swelling and skin ulcers of the second and third left fingers and second right finger. The fingers were abnormally short and the nails were hypertrophic. Roentgenograms disclosed terminal phalangeal resorption in the fingers and toes. Findings were normal from tests for inflammation and dysimmunity. Biopsies of the skin lesions showed fibrosis without inflammation. Neurological evaluation and electromyographic study were normal. Serological tests for syphilis were negative. Calcium and phosphate levels in blood and urine were normal. A diagnosis of idiopathic phalangeal acroosteolysis (Joseph and Shinz disease) was given. This inherited form of acroosteolysis is transmitted on an autosomal dominant or recessive basis. It affects the tips of the distal phalanges of the fingers and toes but occasionally spreads to other bones.     

Surgical treatment of a massive chondrosarcoma in the skull base associated with Maffucci's syndrome: a case report

BACKGROUND: A successfully treated massive chondrosarcoma in the skull base associated with Maffucci's syndrome is presented. The purpose of this report is to discuss the surgical approach to the tumor and reconstruction of the skull base. CASE DESCRIPTION: A 36-year-old woman who had a history of multiple enchondromas and subcutaneous hemangiomas presented with decreased right visual acuity and left papilledema. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a mass in the skull base. The tumor occupied the nasal and paranasal cavities, and extended to the anterior, middle, and posterior intracranial spaces. The midline skull base structures and the left middle cranial base were destroyed. Using a combined anterior craniofacial and left orbitozygomatic approach, the tumor was totally resected. The large skull base defect was reconstructed with a vascularized outer table parietal bone graft attached to a bipedicled temporoparietal galeal flap. The postoperative course was uneventful except for decreased left visual acuity, and temporary diplopia and facial hypesthesia. In 40 months of follow-up there was no recurrence.CONCLUSIONSA skull base approach should be selected to perform total resection of an extensive skull base tumor. The bipedicled temporoparietal galeal flap and vascularized calvarial bone was useful for simultaneous reconstruction.     

Idiopathic testicular infarction: a case report

We herein report a case of idiopathic testicular infarction. The patient was a 20-year-old man with the chief complaint of right testicular swelling and pain. Ultrasonography and color Doppler sonography demonstrated a hypoechoic lesion without any blood supply. Torsion of the right spermatic cord was most suspected, but we could not exclude the possibility of testicular cancer judging from the findings obtained by computed tomography and magnetic resonance imaging. Surgical exploration did not reveal any torsion of the spermatic cord. Subsequently, right high orchiectomy was performed because of the risk of a testicular cancer. Histopathological examination revealed a hemorrhage and congestion of the testis and epididymis.     

Idiopathic adrenal hematoma: a case report

We report a rare case of idiopathic adrenal hematoma. Including our case, 13 such cases have been described in Japan. A 63-year-old [correction of 65] woman was admitted to our hospital for further examination of a right adrenal mass on ultrasonography. Laboratory tests including hormonal assay were within the normal ranges. Computed tomography showed a tumor with calcification measuring 3.0 x 2.0 cm in the right adrenal gland. Magnetic resonance imaging (MRI) revealed a mass with heterogeneous low to iso signal intensity on T1-weighted images and high signal intensity on T2-weighted images. A peripheral rim of the mass was slightly enhanced on dynamic MRI. The patient underwent laparoscopic adrenalectomy. Histopathological examination revealed an old hematoma without neoplastic cells or vascular lesions and these findings were evidence of idiopathic adrenal hematoma.     

Idiopathic abdominal cocoon: report of a case

The abdominal cocoon is a rare disease in which there is total or partial encapsulation of the small bowel by a fibrous membrane. A correct diagnosis is not often made pre-operatively. Awareness of this rare cause of surgical emergency may prevent delay in treatment and avoid unnecessary procedures for the patient, such as bowel resection. We report a patient diagnosed with abdominal cocoon who was subsequently treated.     

Idiopathic sclerosing encapsulating peritonitis: a case report

Sclerosing encapsulating peritonitis is an uncommon but potentially lethal condition, which can lead to severe complications such as bowel obstruction, enterocutaneous fistualas and necrosis. The treatment is debated because of the high morbidity and mortality of the surgical approach. The aim of this study was to contribute to achieving a better approach to surgical treatment of sclerosing encapsulating peritonitis. This report describes an even rarer case of idiopathic sclerosing encapsulating peritonitis diagnosed intraoperatively, observed in the Insubria University Department of Surgery in Varese, Italy, where surgery was mandatory due to the sudden onset of the disease and lack of a definitive diagnosis. Temporary ileostomy and partial resection of the peritoneum were performed. At present (December 2002) the patient is alive and well. Management of sclerosing encapsulating peritonitis is controversial, but the surgical treatment should be as conservative as possible. Guidelines for the treatment of sclerosing encapsulating peritonitis are needed.     

Osteosarcoma of the skull base: a case report

Osteosarcoma, usually observed in long bones, is the second most frequent primitive malignant bone tumor after myeloma. The skull base is an exceptional localization. We report a case of skull base osteosarcoma managed in our department. A 23-year-old female was admitted for bilateral epistaxis, headache, decreasing visual acuity then blindness. Physical examination revealed bilateral blindness and exophthalmia. Cranial magnetic resonance imaging showed a voluminous mass in the skull base extending to the nasosinusal area. A rhinoseptal surgical approach was used but the tumor was so huge that excision was impossible. The biopsy identified at an osteoblastic osteosarcoma. Search for extension (chest computed tomography and abdominal ultrasonography) was negative. Chemotherapy was to be delivered before combination chemoradiotherapy but the patient died before any treatment could be started. Osteosarcoma of the skull base is very rare. The treatment is based on surgery which should be as complete as possible followed by chemoradiotherapy. Prognosis is poor. Median survival is around six months.