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The authors report two cases of lipomatosis. Biochemical study of lipomas revealed, in comparison with normal adipose tissue:--an increase in lipasic lipoprotein activity;--a decrease in lipasic triglyceride activity. These changes could be in part implicated in the mechanisms of fatty accumulation within the lipomas, without explaining the topography and encapsulation.
A case of pelvic lipomatosis is presented and the literature is reviewed. The radiologic signs enable the radiologist to make an accurate diagnosis. This should avoid unnecessary laparatomy.
Benign symmetric lipomatosis (BSL) leads to characteristic multiple lipomas of the neck and submandibullar region. Seven men with BSL, aged 36--65 years, were investigated with regard to alcohol consumption, clinical findings and liver histology. They admitted a long standing ethanol intake (x = 107 ml/day) prior to the development of BSL. The growth of the lipomas was irregular and occurred over a period of months. There was no correlation with overweight or hyperlipidemia. However, liver biopsy revealed features consistent with alcoholic liver disease in all subjects. It is concluded that BSL represents a symptom of alcoholic liver disease affecting middle aged men.
Benign symmetric lipomatosis (BSL), also known as Madelung's disease, is a rare condition and characterized by diffuse but painless growth of unencapsulated lipomas. A close correlation to alcohol and nicotine abuse, metabolic disturbances and malignant tumours have been observed. Surgical treatment is frequently followed by recurrence, nevertheless, it can yield satisfactory functional and cosmetic results. A case of BSL with uncommonly distributed tumors is reported.
Multiple symmetrical lipomatosis, or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and in the upper body. The neoformations grow slowly and their initial consequence is purely esthetic. They can, however, lead to compression of the laryngeal-tracheal area and of the esophagus. This disease normally affects middle-aged males from the Mediterranean area with a history of alcohol abuse. Although most cases have been sporadic, a few authors have indicated that the disorder may be hereditary. It is thought that this pathology originates from an alteration in lipid metabolism. Surgical removal of the lipomatose mass is the treatment of choice even though there are frequently recurrences. A case is presented of a rare laryngeal localization of this disease and diagnosis and treatment are discussed.
Encephalocraniocutaneous lipomatosis (ECCL) is a congenital neurocutaneous disorder of the adipose tissue. The dominant features of the syndrome, include: unilateral cerebral malformation, ipsilateral scalp, face and eye lesions and convulsions beginning in infancy. The authors report a case of 13-year old girl with signs of ECCL syndrome treated surgically for to intractable epilepsy.
We are making a brief review of the literature analyzing the development of epidural spinal lipomatosis and some of the possible causes, additionally, a proposition for alternative therapy is done for those cases that surgery has to be avoided or delayed. Finally, we make reference to some current surgical options.
We describe a patient who initially presented with asymptomatic hydronephrosis. He underwent extensive radiologic evaluation which led to the diagnosis of pelvic lipomatosis. The possible etiology, workup, and treatment options of this unusual entity are discussed.
Aim of this paper is to describe and discuss, on the basis of the available literature, the case of a young woman, previously colectomized for diffuse lipomatosis of the colon, showing hypomagnesemia and symptomatic (precordial discomfort) changes of repolarization phase, detected by ECG, probably due to coronary spasm. This hypomagnesemia (1.4 mEq/1) was probably due to altered intestinal absorption of magnesium, linked to a short bowel syndrome. The ECG changes and the precordial symptom were completely reversed by a relative short treatment with magnesium per os, which increased the magnesium level to low borderline value (1.6 mEq/1). The observation of ECG changes with precordial discomfort, probably linked to hypomagnesemia, suggests the need for routinary magnesium determinations to detect deficiency of this electrolyte, with the scope of improving the diagnosis and the treatment of several symptoms, otherwise difficult to interpret.
A new case of this rarely described pathology is reported. It concerns a benign infiltrating lipomatosis, strictly located to the face. However, dento-musculo-squelettal structures are constantly involved, usually located to a hemiface. A young female patient has been followed up from her birth. Surgical procedures are reported. Diagnostic and therapeutic difficulties of this rare clinicopathologic entity are discussed.
A case of benign, symmetrical lipomatosis is presented. The clinical aspects of this entity are discussed and points of interest in the head and neck are noted.
BACKGROUND: Encephalocraniocutaneous lipomatosis (ECL) involving the scalp and cerebellum was observed without asymptomatic expression. CASE REPORT: A seven-month-old infant presented with two soft subcutaneous hairless tumors of the scalp without any associated clinical anomaly. Neuroradiology explorations (radiography, CT-scan and MRI) showed a lipoma in the cerebellum linked with a occipital cutaneous lipoma through a bone defect. At the age of 3 years, the child remains healthy. DISCUSSION: ECL is a rare neurocutaneous disorder that consists of skin lipomas associated with various cerebral anomalies. ECL may occur as a circumscribed form of the Proteus syndrome, since a few ECL patients have associated manifestations of proteus syndrome as well as localized hypertrophy. However, minor forms of ECL are possible and may be compatible with normal life.
An unusual combination of a fusiform aneurysm on the middle cerebral artery branch to the central region and cortical lipomatosis is described. The etiology of lipomatous disorders in the brain are multiple. The possibility of a combined dysplasia based on maldifferentiation of primitive meninges and the arterial wall is suggested in this case. Treatment consisted in uneventful, microsurgical image-guided resection of the aneurysm using the Surgiscope robotic system.
Pelvian lipomatosis is a proliferative process of fatty tissue in the perivesical and perirectal pelvian space. The etiology is unknown and some authors consider that in reality is a localized form of obesity. Diagnosis can be incidental, or be part of a sequence within a study for unspecific symptoms such as pelvian complains or perineal problems. As part of the presentation of a clinical case report, the authors make a revision of this disease, focusing in several particular aspects, like diagnostic methods, treatment, etc.
Contribution of one case of pelvic lipomatosis, suspected through diagnostic imaging (CAT) and diagnosed by exploratory laparotomy, leading to severe unilateral ureterohydronephrosis and contralateral distal ureteral ectasia. The features of the disease, presentation form, diagnosis and treatment are discussed.
Liposarcoma is the second most common soft tissue sarcoma in adults. These neoplasms take their origin from primitive mesenchymal cells and are rarely encountered in fat rich areas, such as subcutaneous tissue and/or the subserosa of the intestinal tract which, on the contrary, are the two most common sites of lipomas. The two major locations of liposarcomas are the extremities and the retroperitoneum followed with much less frequency by the inguinal region. Other sites are uncommon, particularly the mesentery (9 cases to date in the literature) and, even more so, the mesocolon (only 3 cases of primary sarcoma of the mesocolon reported to date). This paper reports on the case of a well differentiated "lipoma-like" liposarcoma of the sigmoid mesocolon, associated with multiple lipomatosis of the recto-sigmoid colon in a 75 year-old female patient. Surgical treatment consisted of a trans-anal extra-peritoneal anterior resection by CEEA 28 stapler under endoscopic vision. The patient has been followed up for the last 2 years and is still disease-free and well. The peculiarity of the case consists in the contemporaneous presence in close contiguity of two different rare neoplasms whose association is not yet known.
Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome characterized by unilateral scalp, facial, and ocular lesions and ipsilateral cerebral malformations. To define the neuroimaging features of this disorder we studied two patients affected by ECCL and compared our data with those reported in the literature. Sonographic, CT, and MR imaging examinations showed quite specific CNS findings that are highly suggestive of the diagnosis of ECCL. To our knowledge this is the first report of a complete neuroradiologic evaluation and follow-up of this disorder.
Dercum's disease (lipomatosis dolorosa) is a relatively unknown illness. The disorder usually affects middle-aged females. Subcutaneous fatty tissue deposits may occur in many parts of the body. The upper arms, elbows, stomach wall, buttocks, thighs, or knees are most commonly affected. Severe hyperalgesia is found on light pressure and touch. Analgesics or pain-modulating drugs usually have little or no effect. The following case report demonstrates successful symptomatic treatment of the otherwise nearly unbearable complaints: intravenous infusions of 5 mg/kg body weight of lidocaine over 30-90 min may give pain relief lasting several weeks or even months. Alternatively, patients are treated with 150-750 mg orally administered mexiletine daily. Surgical excision or liposuction of these fatty tissue deposits have shown significant reduction of pain. However, this effect reduces over time and recurrences often develop.