Non-encapsulated Lipomas Research Articles

SUN-605 Mediastino-Abdominal Lipomatosis: Deep Accumulation of Fat Mimicking a Respiratory Disease

Introduction: Multiple systemic lipomatosis (MSL) is a rare disorder with unknown etiology. It is characterized by the massive development of non-encapsulated lipomas in subcutaneous tissues. Lipomatosis of the face, head, neck, extremities, abdomen, and pelvis have been reported in the literature. Case: We report a case of a 65 years old female (BMI: 34 kg/m2) with past medical history of hypertension, hyperlipidemia(DLD) diabetes mellitus type 2 and sleep apnea that was brought into emergency room (ER) for worsening shortness of breath. In the ER, she was having an oxygen saturation of 75% and required intubation. The patient was afebrile, with a BP of 120/75 mm Hg and a heart rate of 70/minute. Trans-thoracic echocardiogram revealed normal ejection fraction and normal pulmonary pressure with no wall motion abnormality. CXR showed no infiltration or consolidation. CT angiogram (CTA) ruled out pulmonary embolism but it was notable for large deposits of fat involving the abdomen and thorax, with invasion into the mediastinum and the space between the liver and diaphragm. Mesenteric fat was increased. Tissue was biopsied, which confirmed the diagnosis of fatty invasion. Discussion: Abdominal lipomatosis is characterized by massive enlargement of the abdomen due to intraperitoneal and retroperitoneal fatty deposits. Phenotypically patients can appear to be thin or obese, however, it is more common in the overweight population like our patient. Mediastinal lipomatosis is a benign cause of mediastinal widening, however patients can develop respiratory symptoms like exertional dyspnea due to compression of airways. MSL affects white caucasian between 25-60 years old and it is associated with DLD, impaired glucose tolerance, hyperuricemia, macrocytic anemia, and peripheral neuropathy. The pathophysiology is not fully understood, some theories stated that it is related to defective lipid mobilization in lipomatocytes, other suggested disorder in the mitochondria of brown fat. Conclusion: CT and MRI of the abdomen and chest are very helpful in the diagnosis of MSL but a tissue biopsy is what makes the final diagnosis. There is no definitive treatment; the recommendations are a healthy lifestyle including a low-fat diet, abstinence from alcohol and exercise. In severe cases, surgery is recommended.

Complications of the surgical excision of encapsulated versus nonencapsulated lipomas: A retrospective analysis

Complications of the surgical excision of encapsulated versus nonencapsulated lipomas: A retrospective analysis

Symmetrical Lipomatosis of the Tongue: a Case Report

Multiple symmetric lipomatosis (MSL) is rare and characterized by diffuse growth and non-encapsulated lipomas. The condition is uncommon in the oral and maxillofacial regions, and the incidence is thought to be between 1% and 5%. An 87-year-old male patient with rheumatoid arthritis was referred to our clinic for evaluation of tongue lesions. Intraoral findings showed multiple yellowish submucosal nodules on both of the lateral borders of the tongue, with fibrous consistency, and covered by intact mucosa. Our clinical diagnosis was symmetrical lipomatosis of the tongue. Thus, one of the tongue nodules was biopsied. Histopathological examination revealed non-encapsulated lobules of mature adipocytes with slight variation in size and shape, characterizing a lipoma. The final diagnosis was tongue MSL. Currently, the patient is under clinical follow-up by our team.

Characterization of two distinct lipomas: a comparative analysis from surgical perspective

Background: Lipomas are common benign soft tissue tumors that are well-circumscribed and encapsulated. However, adipose masses that are not demarcated from the surrounding fat are often encountered. Two distinct types of lipomas were analyzed from surgical perspective.Methods: Thirty patients were enrolled after lipoma excision and diagnosed with either encapsulated (n = 20) or non-encapsulated lipoma (n = 10). Comparison of clinical variables, histologic analyses and characterization of the lipoma adipose-derived stem cells (ASCs) between the two lipomas were performed.Results: Non-encapsulated lipomas were associated with older age at operation, larger tumor and increased seroma formation. The density of lymphatic vessels and gene expressions related to lymphatic vessel, inflammation and proliferation were increased in non-encapsulated lipoma. ASCs of non-encapsulated lipoma showed enhanced proliferation when cultured with serum.Conclusions: Non-encapsulated lipomas and their ASCs showed distinct lymphatic histology and cellular response. These findings elucidated the pathogenesis and pathophysiology of lipomas.

Symmetrical lipomatosis of the tongue: Case report and literature review

Multiple symmetric lipomatosis is rare and characterized by diffuse growth and nonencapsulated lipomas. It is usually found in the posterior neck and upper trunk, and the entity is known as “benign symmetric lipomatosis,” “Madelung disease,” and “Launois-Bensaude syndrome.” Symmetric lipomatosis of the tongue was first described by Desmond and is an extremely rare condition. A 74-year-old man complained of painless tongue swelling and difficulty speaking. Clinical findings revealed no tumor masses on the trunk, limbs, or head and neck region. Intraoral findings included soft yellowish masses with a smooth surface without erosions on the side of the tongue bilaterally. They were 30 mm in diameter. An incisional biopsy was taken from the mass, and the lipoma was diagnosed. The bilateral tongue lesions were resected under general anesthesia. Intraoperative findings revealed adipose tissues interspersed with lingual muscles and no capsulation. The lesion was finally diagnosed as symmetric lipomatosis of the tongue based on clinical findings and radiological and histologic examination.

Multiple symmetric lipomatosis as a genetic disorder: a review

Multiple symmetric lipomatosis (MSL) is a rare disorder characterized by the presence of multiple, symmetric nonencapsulated fat masses in the face, neck, and other areas. The typical presentation of MSL consists of massive lipomatous deposits around the neck which has given rise to the classic description of a buffalo hump or horse collar. The etiology is unknown although autosomal dominant or mitochondrial inheritance has been postulated based on several affected families and tissue studies. We reviewed the current literature and present a 43-year-old male with a circumferential neck mass composed of multiple nonencapsulated lipomas. His family history is unique with eight family members over three generations.

Mesotherapy for Benign Symmetric Lipomatosis

Benign symmetric lipomatosis, also known as Madelung disease, is a rare disorder characterized by fat distribution around the shoulders, arms, and neck in the context of chronic alcoholism. Complete excision of nonencapsulated lipomas is difficult. However, reports describing conservative therapeutic measures for lipomatosis are rare. The authors present the case of a 42-year-old man with a diagnosis of benign symmetric lipomatosis who had multiple, large, symmetrical masses in his neck. Multiple phosphatidylcholine injections in the neck were administered 4 weeks apart, a total of seven times to achieve lipolysis. The patient's lipomatosis improved in response to the injections, and he achieved good cosmetic results. Intralesional injection, termed mesotherapy, using phosphatidylcholine is a potentially effective therapy for benign symmetric lipomatosis that should be reconsidered as a therapeutic option for this disease.

Benign symmetric lipomatosis (Madelung?s disease) complicated by involvement of the facial nerve

Benign symmetrical lipomatosis (Madelung’s disease) is a rare disease of unknown etiology which is characterized by the diffuse growth of non-encapsulated lipomas, usually on the neck and upper trunk. Given the benign nature of the lesion, surgical debulking is the treatment of choice, with liposuction reserved for smaller lesions. A 60-year-old man presented in our clinic with progressive tumor growth especially in the cheek. Intraoperatively all branches of the facial nerve were found to be covered by the lipoma. Although Madelung’s disease is a benign lesion, the location of lipoma with possible involvement of vital structures may influence the choice of treatment.

Multiple Symmetric Lipomatosis (Madelung's Disease) Caused by the MERRF (A8344G) Mutation

Multiple symmetric lipomatosis (MSL) was first described by Brodie in 1846 and is characterized by accumulation of non-encapsulated lipomas in the cervical-cranial-thoracic region. Alcohol consumption is regarded as essential in lipoma development by some. Mitochondrial dysfunction was first reported in 1991. Since then, there has been controversy regarding etiology of MSL, with a number of studies supporting and some refuting the role of mitochondrial dysfunction. We report on 2 cases of MSL with pathologic (ragged-red fibers) and molecular (A8344G mutation) features of mitochondrial dysfunction. A literature review revealed that mitochondrial gene dysfunction was evident in 28% of MSL cases. Furthermore, the MERRF mutation (A8344G) was detected in 16% and mitochondrial gene deletions in 12% of MSL cases. Therefore, clinicians need to be vigilant of the fact that a significant proportion of the MSL phenotype results from mitochondrial gene mutations and/or deletions.

Combined surgical lipectomy and liposuction in the treatment of benign symmetrical lipomatosis of the head and neck.

Benign symmetrical lipomatosis (Madelung syndrome) is a rare disease of unknown aetiology, which is characterised by diffuse growth of non-encapsulated lipomas. Between 1995 and 2000 we treated 11 patients with benign symmetrical lipomatosis in the head and neck. The group comprised 10 men and one woman aged 34 to 62 years (mean 47). The most common complaints were reduced range of movement of the head and obstruction when eating or speaking. Combined lipectomy and liposuction were done for all patients, with liposuction being done at a second session. The mean follow-up period was 2.7 years. The functional results were satisfactory in all patients. Nine of the 11 patients were also satisfied with the aesthetic outcome. Two patients developed recurrence 1.5 and 2 years after the operation, respectively. There were no serious complications. We think that combined lipectomy and liposuction is a successful procedure for treating benign symmetrical lipomatosis in the head and neck region. Nevertheless, the advantages and drawbacks of the two techniques should be considered preoperatively.

Doença de Madelung: relato de casos e revisão da literatura

A doença de Madelung caracteriza-se pelo acúmulo simétrico de massas de tecido lipomatoso, não-encapsulado, na região cervical e tronco superior (tipo I; mais comum) ou de forma difusa, como uma simples obesidade (tipo II). Constitui uma entidade benigna rara, de etiologia desconhecida, porém com uma clara associação com o abuso de álcool. É mais freqüente em homens de meia idade e descendentes de italianos. Os autores apresentam 3 casos de Lipomatose Simétrica Múltipla, classificados como tipo I, que referiam deformidade estética e diminuição da mobilidade cervical, sem queixas de compressão do trato aerodigestivo. Os pacientes foram tratados cirurgicamente, com bom resultado estético e sem complicações perioperatórias, comprovando a eficácia da abordagem cirúrgica. Também fazem uma revisão da literatura.

Multiple symmetric lipomatosis

Multiple symmetric lipomatosis (MSL) is a rare disorder of middle life characterized by large nonencapsulated lipomas distributed around the neck, shoulders, and other axial regions. Neurologic involvement, particularly peripheral neuropathy, is frequent. The pathogenesis of the syndrome is still unknown, but ragged-red fibers are occasionally present in muscle of affected patients, suggesting a mitochondrial abnormality. We studied 11 unrelated patients with MSL by means of neurophysiology, muscle morphology, muscle biochemistry, Southern blot, and PCR analysis of mitochondrial DNA. All patients were men aged 41 to 63 years. Clinical or electrophysiologic signs of a sensorimotor polyneuropathy were present in nine patients, eight of whom had a history of alcoholism. In muscle biopsy specimens, the most prominent feature was pathologic subsarcolemmal aggregates of mitochondria. Biochemical analysis of respiratory chain enzymes revealed a moderate but significant decrease of cytochrome c oxidase activity as compared with age-matched controls. In one patient, Southern blot analysis showed multiple deletions of mitochondrial DNA. We conclude that mitochondrial dysfunction is common in MSL and may be based on identifiable defects in the mitochondrial genome.