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Paraspinal lipomatosis usually occurs in patients with steroid excess. In two patients a paraspinal mass was detected on conventional radiographs and was subsequently shown to represent benign fat deposition on computed tomography, thereby obviating further studies. Neither patient had been receiving steroids. Paraspinal lipomatosis may be the cause of a benign mass and may be considered in the differential diagnosis of this finding.
The authors report six new cases of epidural lipomatosis--a rarely described entity characterized by increased extradural fat within the spinal canal causing compression of the spinal cord and neurologic deficits. Five of the six patients had a history of chronic steroid use. Myelography of the thoracic spine, followed by computed tomography (CT), was performed in five patients, and magnetic resonance (MR) imaging was done in three. Myelographic findings were abnormal in three patients and normal in two, compared with those of postmyelography CT, which revealed increased accumulation of extradural fat in all five patients. In the three patients in whom it was performed, MR imaging also showed the increased spinal canal fat. Four patients underwent surgery, which revealed an increased amount of histologically normal, unencapsulated fat in all cases. The diagnosis of epidural lipomatosis should be based on a combination of clinical, imaging, surgical, and histologic findings. If the clinical suspicion is high, even if results of myelography are normal, further evaluation with post-myelography CT or MR imaging should be considered.
We report a relatively rare case of renal replacement lipomatosis presenting as a renal mass. Computed tomography revealed a predominantly low-density and roundish mass, with an irregular renal parenchyma, high-density calcification, and abundant low-density fat. The differential diagnosis before surgery was squamous cell carcinoma, teratoma, or angiomyolipoma of the kidney. The case was initially misdiagnosed, because we had no experience with this disease. After mass exploration, histological examination confirmed the diagnosis of renal replacement lipomatosis. The patient was free from signs of recurrence 10 months after the operation.
A case of epidural lipomatosis in a 49-year-old man presenting with paraparesis, midthoracic pain, and Staphylococcus aureus pneumonia is reported. The patient had been on low dose corticosteroid therapy for 7 years for rheumatoid arthritis. The clinical and myelographic findings suggested a diagnosis of epidural abscess, but the only abnormality discovered at operation was abundant fatty tissue in the dorsal epidural space significantly compressing the spinal cord, and this was partially removed. Postoperative neurological improvement suggested that the lipomatosis was responsible for the spinal cord compression and dysfunction. If this diagnosis had been suspected, it might have been confirmed by magnetic resonance imaging or postmyelography computed tomographic scanning. With such a diagnosis, an alternative treatment could have been to decrease the steroid dose, observe for clinical improvement, and perhaps avoid operation.
We report a case of extensive renal replacement lipomatosis demonstrated by ultrasound (US) and computed tomography (CT) in a 57-year-old woman with a history of two cadaveric renal transplants. One transplant was non-functional due to chronic rejection. The second renal transplant is functioning normally and the renal replacement lipomatosis did not cause mass effect on either of the renal transplants.
We report a case of systemic intraosseous lipomatosis involving the proximal femur, both ends of the tibia, and the tarsal and metatarsal bones. The lesions progressed during a five-year follow-up with a pathological fracture of the tibial plateau. CT scans were characteristic and helpful in diagnosis but MR imaging added little information. Intraosseous lipomatosis is a hamartomatous malformation due to hyperplasia of adipose tissue, and is fundamentally different from solitary benign intraosseous lipoma. Management involves reconstruction of any pathological fracture. Large progressive lesions should be treated by curettage and grafting in an attempt to prevent such fractures.
We report a case of spinal epidural lipomatosis (SEL) caused by ectopic Cushing's syndrome and give a review of the literature. The most common cause of SEL is prolonged therapy with glucocorticoids, only a very few cases are related to endogenous Cushing's syndrome. The pathophysiological mechanism is not clear but there is a possible role for the autonomic nervous system in the stimulation of growth of epidural fat. Severe neurological symptoms which indicate myelopathy and radiculopathy can occur, but there is often a delay in diagnosis because the non-specific initial symptoms are not recognized. The epidural fat is mostly located in the thoracic and lumbar region. Magnetic resonance imaging can establish the diagnosis rapidly. In patients with severe neurological symptoms, surgical decompression of the myelum and removal of the epidural fat is the treatment of choice. Most patients have partial or complete recovery of neurological deficits after surgical treatment or after discontinuing glucocorticoid therapy; mild cases can also be treated conservatively. Routine imaging for the detection of epidural-located lipomatosis in patients at risk is probably useful.
Spinal extradural lipomatosis is rare and is usually associated with long-term steroid administration or obesity. It is most commonly thoracic in situation. We present a unique case of spinal extradural lipomatosis in a 20-year-old non-obese, clinically normal man. The role of MRI in investigation and the management options are discussed.
The authors describe a female patient with unilateral malformations of skin, cerebrum and eye. The symptoms consisted of local skin hypoplasia, skin appendages and lipomatous tissue; cysts, hypoplasia and lipomatosis of the brain; and ocular malformations. In the newborn period the symptoms led to the diagnosis of oculocerebrocutaneous (OCC) syndrome. In the first year of life the clinical course deteriorated and the psychomotor development was progressively retarded. Evaluation at the age of 15 months prompted the authors to change the diagnosis to encephalocraniocutaneous lipomatosis (ECCL). The differential diagnosis of ECCL and OCC syndromes is discussed and a possible common pathogenetic pathway of these two rare disorders is proposed.
Patients with Cushing's syndrome may develop spinal epidural lipomatosis, an abnormal accumulation of fat in the spinal epidural space. This accumulation of fat may cause compression of the spinal cord or cauda equina with resulting neurological deficit. Two cases of symptomatic spinal lipomatosis are reported in cardiac transplant patients receiving chronic corticosteroid treatment. The literature is reviewed, and diagnostic and therapeutic considerations are discussed.
A rare case of a segmental small intestinal (jejunal) lipomatosis is described. A 33-year-old male was admitted with a clinical diagnosis of an acute intestinal obstruction. A plain erect abdominal x-ray showed multiple air-fluid levels. On an exploratory laparotomy, a jejunojejunal intussusception was found secondary to a segmental submucosal lipomatosis. This was treated by a segmental resection and anastomosis, which resulted in a complete cure. Here we present this case with a review of the relevant literature.
A man aged 51 for the last 3 months had displayed general malaise, epigastric pain, nausea, vomiting and constipation. Also, he had a pseudo-athletic appearance with symmetrical large accumulations of fat on the front of the trunk, the lower back, the shoulders and the proximal extremities, characteristic of 'benign symmetrical lipomatosis'. He died of embolism of the aortic bifurcation and autopsy revealed an extensive adenocarcinoma in the upper abdomen, probably originating from the pancreas or the stomach. Benign symmetrical lipomatosis mostly occurs in middle-aged men. The pathogenesis is unknown. Association with alcohol abuse, metabolic abnormalities, polyneuropathy and certain malignancies has been described. Treatment is symptomatic by surgery or liposuction.
Pelvic lipomatosis is a rare disease of unknown etiology characterized by benign proliferation of fat in the pelvis. We describe a 27-year-old man with pelvic and retroperitoneal lipomatosis causing a severe urinary and fecal obstruction. The diagnosis was evaluated by barium enema, intravenous urogram, computerized tomography and magnetic resonance imaging. The therapeutic approach is described and discussed.
Two cases of spinal epidural lipomatosis (SEL) were reported. Patient 1 was on oral corticosteroid and patient 2 was obese and had prostate cancer. Patient 1 was a 45-year-old man diagnosed as sarcoid myelopathy at C 5/6 vertebral body levels and had been placed on oral corticosteroid therapy for 14 months. He showed spastic paraplegia, hypesthesia below C 4 level with distal dominancy and dysesthesia below Th 6 level. MRI revealed epidural lipomatosis from Th 3 to Th 9 vertebral bodies, which presented high in T 1 weighted image (WI) and T 2 WI, and non-signal in STIR image. On axial image spinal cord was compressed by this mass. Patient 2 was a 73-year-old man with spastic paraplegia, and superficial and deep sensory disturbances below Th 6. He had been obese (BMI 26.1) upon admission. He was diagnosed as prostate cancer with bone metastasis. On MRI of the thoracic spine revealed epidural mass of high in T 1 WI and T 2 WI, and non-signal in STIR image. SEL is a rare condition known as hyperplasia of normal fat tissue in epidural space which sometimes compresses the spinal cord or spinal nerve roots resulting in neurologic deficit. SEL should be kept in mind as having possible neurologic complications in obese patients or ones on long term steroid therapy.
(1) Lipomatosis (development of non encapsulated fatty masses) is a rare complication of chronic systemic steroid therapy. (2) Epidural and mediastinal lipomatosis due to steroid therapy carries a risk of symptomatic compression. (3) A dose reduction or, if possible, cessation of systemic steroid therapy almost always improves or removes the symptoms.
Corticosteroid injections into the spinal epidural space are frequently used to effect a relief of back pain and associated radicular extremity symptoms. Spinal epidural lipomatosis has been documented after the use of systemic corticosteroid therapy. This case report documents the development of epidural lipomatosis after the administration of multiple epidural steroid injections. The development and subsequent resolution after discontinuation of the steroid injections are demonstrated with serial magnetic resonance imaging.
A case of a small-bowel schwannoma with diffuse familiar lipomatosis is described. This case underlines the rarity of the neoplasm and its probably chance association with subcutaneous lipomatosis. The intestinal neoplasm was diagnosed preoperatively by upper gastrointestinal endoscopy and a small-bowel enema; computed tomography scan confirmed the intestinal lesion. Attention is focused on the morphological features of intestinal schwannomas and their biological behaviour.
Epidural lipomatosis is a rare disease, most often associated with chronic administration of exogenous steroids. We report the case of a 69-year-old man treated with 20mg prednisolone daily for 11 years because of polyarthritis. The patient complained of back pain associated with severe weakness of his lower limbs and brisk reflexes, with a progressive onset over one year. MRI showed epidural lipomatosis narrowing the epidural spaces from T-3 to T-10. Tapering of steroids was associated with slow but positive resolution of his symptoms.