1/21. Spontaneous hemomediastinum complicating steroid-induced mediastinal lipomatosis.Spontaneous hemomediastinum is a rare event, occurring in association with bleeding disorders, intratumoral bleeding, or following an abrupt increase in intrathoracic pressure. We report the case of a patient with systemic lupus erythematosus, nephrotic syndrome, and renal failure, in whom mediastinal lipomatosis (ML) developed following increased corticosteroid therapy. Anticoagulant therapy likely precipitated a massive spontaneous hemomediastinum secondary to diffuse hemorrhage of mediastinal fat, which required emergency decompressive surgery. Steroid-induced ML is common and usually well tolerated, but clinicians should be aware of its potential risk of bleeding when associated with anticoagulant therapy. This case further emphasizes the bleeding complications of treatment with low-molecular-weight heparin in patients with renal failure.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/21. Orbital lipomatous haemangiopericytoma: an unusual variant.Haemangiopericytoma (HPC) of the orbit is a rare tumour occurring in all age groups and presenting with slowly progressive proptosis, ocular motility impairment and visual loss. Although most are benign tumours that are resectable at diagnosis, one-third of these tumours demonstrate malignant features and recur locally or metastasize, usually with a fatal outcome. In 1995, a morphologically unique variant, termed lipomatous haemangiopericytoma (L-HPC), was described. Only one case has been reported previously arising in the orbit. In this paper, a case is reported of L-HPC occurring in the orbit and consider the prognostic implications of this HPC variant.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/21. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.We describe a white female infant with neurocutaneous melanosis (NCM) and encephalocraniocutaneous lipomatosis (ECCL). Multiple, giant and small congenital melanocytic nevi (CMN) were observed on the head, neck and trunk and involved 70% of body surface area. Histologic examination of several CMN revealed atypical nodular proliferations of dermal nevomelanocytes. In a small (<1 cm) truncal CMN, single and dyscohesive intraepidermal nests of atypical nevomelanocytes simulating a superficial spreading melanoma, were observed. The placenta was grossly normal and histologically demonstrated multiple banal appearing nevomelanocytes within the stroma of its villi. At the 17-month follow-up no evidence of primary or metastatic melanoma was present. This previously undescribed association of NCM, ECCL and placental nevomelanocytes provides strong support for the hypothesized causal role of anomalous neural crest morphogenesis and migration in the development of all three disorders. The genetic mechanism underlying these complex birth defects has been hypothesized to result from the action of lethal autosomal dominant genes surviving by mosaicism.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
4/21. nevus lipomatosus cutaneus superficialis.nevus lipomatosus cutaneus superficialis (NLCS) is a rare developmental anomaly characterized by isolated ectopic mature adipose tissue in the dermis. We describe a child with a NLCS present from birth that was growing in size. The lesion was removed by simple excision and has not recurred.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/21. Colo-colic intussusception due to intestinal lipomatosis.Lipomas of the small intestine are usually found incidentally; symptoms occur in less than one-third of affected patients, especially when lipomas are more than 2 cm in size. We report a 32-year-old man in whom intestinal lipomatosis resulted in colo-colic intussusception. These symptoms disappeared following operative resection of about 60 cm of ileum and ascending colon.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/21. Macrodystrophia lipomatosa: a reconstructive approach to gigantism of the foot.Localized gigantism poses a challenging surgical dilemma, and it may be treated with amputation. This case report documents the application of a reconstructive approach to a severe case of pedal macrodystrophia lipomatosa in a 1-year-old girl. A series of 3 surgeries were designed to reduce the length, width, height, and overall bulk of the congenitally enlarged foot. The 3 procedures debulked the foot for normal ambulation and same-size shoe wear for both feet. The resulting functional and aesthetic improvements achieved through reconstructive treatment provided a desirable alternative to amputation.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/21. Crossed renal ectopia with pelvic lipomatosis: a new syndrome involving chromosome 1.An 18-year-old male patient is described who possesses both kidneys on one side (crossed renal ectopia), together with pelvic lipomatosis. In general, lipomatosis is benign, but here the tissue shows the rare feature of malignancy. Chromosomally, the patient is typically characterised by somatic translocations involving chromosome 1 (37% metaphases); these almost always exhibit a whole chromosome translocation with chromosome 6 (35%), although involvement of chromosome 1 with chromosome 8 is present in 2% of metaphases. Other chromosomal features encountered in Giemsa-stained and G-banded preparations from lymphocyte cultures include the prevalence of a small y chromosome in 25% of metaphases, the presence of marker dots in 20%, and acrocentric associations in 8%-10% of metaphases. However, more than 50% of metaphases have a normal 46XY karyotype with a normal-sized Y-chromosome. Crossed renal ectopia with pelvic lipomatosis can therefore be assigned to a new syndrome characterised by a whole-chromosome translocation involving chromosomes 1 and 6.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/21. Idiopathic spinal epidural lipomatosis.Spinal epidural lipomatosis is a rare disorder characterized by the pathological overgrowth of epidural fat and often causes extradural compression which may mimic other spinal disorders. Steroid administration is a well-documented etiology. We report a case of spinal epidural lipomastosis without history of steroid administration. The initial manifestation was bizarre and was misdiagnosed as a degenerative vertebral or disc disease for long. Posterior decompression with debulking of the extradural fat successfully and thoroughly relieved his symptoms. We emphasize the importance of taking this disease into consideration for a markedly obese patient with persisting back pain or symptoms suggesting spinal cord or other spinal neural elements compression.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/21. Recurrent diffuse lipomatosis of the neck--a case report.Diffuse lipomatosis is a rare disorder of adipose tissue occurring in young people . It has a predilection for trunk and proximal extremities where it presents as poorly circumscribed overgrowth of fatty tissue. Definite diagnosis is established by histological examination of tumor. Though it attains extensive size and has a high tendency to recur, the clinical course is benign.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/21. Mandibular osteoma in the encephalocraniocutaneous lipomatosis.INTRODUCTION: Encephalocraniocutaneous lipomatosis is a rare congenital disorder characterized by an abnormal development of adipose tissue, and is of unknown pathogenesis. Catherine Haberland and Maurice Perou first diagnosed this disorder in 1970. To our knowledge, approximately 25 patients have been reported with this diagnosis but so far, only 1 patient has been reported in poland by Roszkowski and Dabrowski in 1997. At that time she was a 13-year-old girl, who was neurosurgically treated. CASE REPORT: The authors followed the same patient (now 21 years old), who was referred to the Department of Cranio-Maxillofacial Surgery of the Medical University of Lodz for osteoma of the mandible. Partial resection of the mandible was performed on account of the size of the lesion. The mandible was reconstructed by an iliac crest bone graft fixed by 2 titanium plates. The surgical procedure is described. CONCLUSION: Encephalocraniocutaneous lipomatosis is a very rare syndrome. Most of the cases described in the literature presented lipomas of the skin with neurological and ophthalmological disturbances. Only a few authors described jaw tumours and no information was found on such big osteomas to this syndrome.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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