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Familial multiple lipomatosis is a rare hereditary syndrome with a proposed autosomal-dominant inheritance. A case of an 89-year-old man with this disease is presented, along with his pedigree. Clinical features, genetic evidence, and treatment options are reviewed.
A juvenile tumour from granulosa cells (M-8622/1), 13 x 8 x 6 cm, in the right ovary in a three-month-old girl produced some symptoms of pseudopubertas praecox isosexualis which disappeared after operation. Microscopic examination of the tumour revealed in addition to typical structures a less common differentiation to Sertoli cells. Despite actinotherapy and chemotherapy one and a half years after the onset of the disease X-ray examination revealed metastases in the lungs which were successfully cured by further doses of the above two types of treatment. Between the age of 6 and 15 years the girl developed successively polyposis of the stomach, small and large intestine (M-7564/0), subcutaneous lipomatosis of the trunk and left lower extremity (M-8881/0) and nodular goitre (M-7164/0), predominantly quiescent. In the literature a connection between gonadal stromal ovarian tumours and mesenchymal tumours, intestinal polyposis and disorders of the thyroid gland is described, but in different patients. The authors' observation is unique by the successive incidence of these changes in a single patient surviving 15 years after operation; and thus genetically conditioned associations could be involved.
We report a 52-year-old man with left-sided ulcerative colitis for 5 years and pulmonary sarcoidosis diagnosed 3 years back. He presented with subcutaneous lipomatosis and a right iliac fossa mass, which was diagnosed histologically as appendiceal adenocarcinoma. He was treated with right hemicolectomy, followed by chemotherapy.
In this paper, the preliminary results regarding the alterations of the biological action mechanism of insulin in several types of pathological accumulations of fat, i.e. lipomas, Madelung's Lipomatosis and liposarcoma, are presented. The results indicate significant alterations both at the insulin receptor and post-receptor levels, with reduced biological activity of this hormone, which could have and inductive role in the pathogenesis of such entities.
Spinal epidural lipomatosis is most commonly observed in patients receiving long-term exogenous steroid therapy, but can also be seen in patients with endogenous steroid overproduction, obesity, or idiopathic disease. With this condition, there is hypertrophy of the epidural adipose tissue, causing a narrowing of the spinal canal and compression of neural structures. A majority of patients will present with progressive myelopathy, but radicular symptoms are also common. Conservative treatment--weaning from steroids or weight loss--can reverse the hypertrophy of the adipose tissue and relieve the neural compression. If conservative management fails, surgery with decompressive laminectomy is also very successful at improving the patient's neurological symptoms.
We report a patient with multiple symmetrical lipomatosis, which is an unusual subgroup of lipomatous disease. This entity is described along with a brief review of the literature on this disease and associated disorders.
The authors report a case of encephalocraniocutaneous lipomatosis (ECCL) in a female infant. Clinical manifestations in this case showed significative overlap with another neurocutaneous syndrome, namely oculocerebrocutaneous (OCC) Delleman's syndrome. This may support the theory of somatic mosaicism. The patient underwent a cerebrospinal fluid (CSF) shunt procedure due to marked tetraventricular hydrocephalus that was not present at birth. The need for close and multidisciplinary follow-up of these patients is stressed.
We report a 22-year male who developed progressive distension of abdomen, clinically diagnosed as ascites. A diagnosis of abdominal lipomatosis was made on the basis of CT evidence of excessive fatty tissue in abdominal cavity which was confirmed on laparotomy.
Pelvic lipomatosis consists of an abdominal capsulated mass containing lipidic tissue, generally with remarkable dimensions, responsible for urinary tract disturbances. Here we describe the first case to our knowledge of accidental intraoperative diagnosis associated to an ovarian cyst in absence of objective symptoms and signs.
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.
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.
OBJECTIVE: To explore the clinical feature of benign symmetric lipomatosis (BSL) so as to improve its diagnosis and treatment. METHOD: 28 patients of BSL treated in our hospital were analyzed and literature was reviewed. Surgical management is used in all patients. RESULT: There were no patients died. All patients were satisfactory with their appearance and function improved. 28 patients were followed up for 2-15 years (average 6 years and 5 months), only 5 cases recurred. CONCLUSION: BSL usually occurs in middle age men. Patients have a striking appearance "hump back" because of a diffuse, symmetric distribution of the lipomatous tissue in the cervical region. The etiology is related to alcohol abuse. Operation is the most effective treatment. The radical surgical therapy should not be emphasized because the important anatomic structures may be damaged. Abstaining from alcohol may help to reduce the rate of recurrence.
Madelung's disease is a rare disfiguring lipomatosis of unknown etiology. The only effective therapy is the palliative surgical removal of the fatty tissue by lipectomy or liposuction. Experience with eighteen surgically treated patients shows that despite a high rate of complications and the tendency to recur, both techniques might be very useful.
Benign symmetric lipomatosis is a rare disease that predominantly affects males. A close correlation with alcohol and nicotine abuse, metabolic disturbances and malignant tumours of the upper airways has been observed. We report the case of a 61-year-old female patient whose first clinical lesions had appeared more than 30 years earlier. The huge enlargement of fatty tissue had also involved her face. Case history, laboratory findings and radiological and ultrasound examinations allowed us to exclude metabolic disturbances and associated diseases.
Benign symmetric lipomatosis (BSL) is a rare proliferative disorder of the adipose tissue, characterized by symmetrical fat deposits, predominantly in the neck and shoulder area, upper back and arms. This study contains the analyse of a lot of ten patients with BSL, which were surgical treated in our department between 1994-2004. The followed parameters were: sex, age, heredo-collateral antecedents, associated organic diseases, the motivation of medical addressability, the sites of first lesions, the length of evolution before the admission, methods for diagnosis, number of surgical sessions necessary to extirpation of fat deposits, the results of the periodic follow-up examination. The followed patients with BSL were treated by surgery (extirpation of the cervical fat masses): one surgical session (8 cases) or two sessions (2 cases). The morphological, functional and psychic results were good. Recurrence at the site of surgery was not detected in any follow-up subjects. The elective treatment in BSL is surgical, by extirpation of the lipomatous masses; a satisfactory, lasting result can be expected only after the most radical possible extirpation (not only for cosmetic intentions).
A 76-year-old female patient is presented who suffered from muscular weakness in arms and legs. She was obese and had a symmetric accumulation of fatty tissue with a bumpy structure at both arms which gave the patient a pseudoathletic appearance. Fatty tissue accumulations were present at both shoulders, arms, at both thighs, at the back and the abdomen. She suffered from benign symmetric lipomatosis (BSL), also called Launois-Bensaude syndrome (LBS), which is a rare disorder of unknown origin and poorly understood pathophysiology. It is believed to be a disease of disturbed lipogenesis induced by catecholamines. The syndrome is often associated with features of metabolic syndrome such as diabetes mellitus, hyperuricemia, hyperlipidemia and hypertension and is associated with polyneuropathy which is an integral part of the disease. Therapeutic options are pharmacological treatment with salbutamol and surgical procedures such as lipectomy or liposuction.
Multiple symmetric lipomatosis (MSL) is a rare disorder only mentioned in about 200 cases in the medical literature. It manifests as massive lipomatous deposits in specific areas of the body. The cause is unknown, although there frequently is a history of alcoholism. Surgical lipectomy has so far been the choice of treatment. We present a review of the disease and report one case successfully treated with liposuction.
The description is given of Shwachman syndrome in a male of 17, with a long anamnesis of malabsorption syndrome, granulocytopenia, repeated bronchopneumonia, somatogenic subnanism and hypogonadism. The death resulted from acute cardiovascular insufficiency. Post-mortem examination revealed exocrine pancreas hypoplasia with lipomatosis, Wirsung duct atresia, fibrocholangiocytosis and fat hepatosis, visceral anemia, delay in the maturation of granulocytes in the bone marrow, somatogenic polyglandular endocrinopathy, metabolic myocardium necrosis, calcinosis of the small arteries wall, lymphoid tissue atrophy, focal pneumonia of the upper lobe of the left lung.