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Investigation of radiosialadenitis during fractioned irradiation: sialoscintigraphical and histomorphological findings in rats.

Xerostomia is the most debilitating side effect induced by irradiation of head and neck tumours and is caused by irradiation damage to the salivary glands. The aim of this study was to correlate structural histomorphological damages and sialoscintigraphical findings during fractioned radiotherapy. The head and neck area of 27 WAG/RijH rats was irradiated with 60Co-gamma rays (60 Gy/30f 6 weeks). To evaluate salivary gland function, a port system was implanted, and 99mTc-pertechnetate was applied at different stages of irradiation (0, 16, 30, 46, 60 and 6 months post-irradiation). In the course of treatment the parotid glands were examined histopathologically. Rat salivary glands developed a dose-dependent radiosialadenitis. After a dose of 16 Gy an intra- and extra-cellular oedema developed in the salivary glands. Progressive vacuolisation (30 Gy) developed into lipomatosis (46 Gy) and necrotic changes (60 Gy) in the parotid glands. Six months after irradiation treatment, the chronic histomorphological damages corresponded to stage II according to Seifert. The corresponding loss in gland function investigated by measurement of the 99mTc-pertechnetate uptake of the salivary glands was 13% (16 Gy), 26% (30 Gy), 57% (46 Gy), 75% (60 Gy) and 66.5% (6 months post-irradiation). The presented animal model is suitable to demonstrate the correlation of histomorphological and sialoscintigraphical findings.

Animals↗

The UV hand lamp as a helpful instrument for macroscopic visualisation of myocardial fibrosis during autopsy.

The application of ultraviolet light in forensic practice is a well-tested method to visualise traces or skin alterations. When viewing myocardial sections under UV light at autopsy, bluish-white fluorescent areas became visible that could not be detected in the seemingly homogenous myocardium in daylight. A systematic comparison of the macroscopic cardiac findings and the pertinent histological sections showed that in all cases with a positive UV fluorescence myocardial fibrosis or lipomatosis could be confirmed by histological methods. The examination for UV fluorescence of the myocardium seems to be a simple and rapid method improving the macroscopic perceptibility of fibrotic changes and minor scars.

Adult↗

Epidermal naevus and segmental hypermelanosis associated with an intraspinal mass: overlap between different mosaic neuroectodermal syndromes.

UNLABELLED: The epidermal naevus syndrome is a neurocutaneous syndrome characterised by the association of epidermal naevi with central nervous system, skeletal, ocular or cardiovascular abnormalities. We report on a 1.75-year-old boy who presented with spastic diparesis, a partial paresis of the left forearm and macrocephaly. He had a large epidermal naevus along the cervical spine and a segmental hypermelanosis. MRI studies revealed a large intraspinal mass extending from the lower cervical to the upper thoracic spine. The condition of our patient demonstrates the overlap of the epidermal naevus syndrome with well-defined mosaic neuroectodermal phenotypes such as encephalocraniocutaneous lipomatosis, Feuerstein-Mims syndrome and Proteus syndrome. CONCLUSION: we recommend evaluation of all patients with large epidermal naevi, especially in the head and neck region, for the presence of central nervous system abnormalities or neoplasms.

Cervical Vertebrae↗

Inflammatory synovitis due to underlying lipoma arborescens (gadolinium-enhanced MRI features): report of two cases.

Lipoma arborescens (synovial lipomatosis) is a rare, benign intra-articular lesion of unknown etiology. It is a very rare primary benign tumour of the synovium, which usually affects the knee joint and can lead to slowly progressive chronically swollen knee. In this report we described two cases with this rare entity with initial presentation of inflammatory synovitis. We also described the Gadolinium (Gd-DTPA)-enhanced MRI features. To the best of our knowledge, this is the first report that described inflammatory synovitis in association with this rare entity.

Adult↗

Cystitis glandularis.

We present 12 cases of cystitis glandularis which fall into two distinct groups, one characterized by hematuria and the other by urinary tract infection. We can produce no clinical evidence that this disease is premalignant, but emphasize the necessity of histology to exclude malignancy since, macroscopically, the condition is indistinguishable from mitotic changes. We also describe the association of cystitis glandularis and pelvic lipomatosis.

Adult↗

Lipoplastic lymphadenopathy presenting as an ovarian mass: a case report.

Lipoplastic lymphadenopathy is a pathological condition wherein accumulations of intranodal fat result in lymph node enlargement and may mimic abdominal, pelvic, and retroperitoneal neoplasms, particularly lymphomas. The pathology appears to be different from lipomatosis in that benign, mature adipocytes and lipids are located within lymph nodes, rather than deposited in body cavities. The case of a 49-year-old woman presenting as an ovarian neoplasm is presented as an example of the pathology and its ability to masquerade as other neoplasms. The etiology of lipoplastic lymphadenopathy is unclear although associated causes are suggested. The difficulties of radiological examination of this case and others makes open lymph node biopsy important for the final diagnosis.

Abdomen↗

Late onset primary systemic carnitine deficiency exacerbated by carnitine-free parenteral nutrition.

We describe a 21-year-old male with previously normal plasma total and free carnitine levels who developed a deficiency manifest by decreased plasma and muscle total and free carnitine, decreased urine carnitine, severe hepatic steatosis, mediastinal lipomatosis, progressively impaired triglyceride clearance, myopathy and intermittent hypoglycemia. This case demonstrates that systemic carnitine deficiency may occur in some patients receiving long term carnitine-free TPN. Carnitine may be an essential element of the diet in this patient population.

Journal Article↗

Long-term follow-up (12 to 35 weeks) after dynamic cardiomyoplasty.

OBJECTIVES: To obtain information on the long-term effects of dynamic cardiomyoplasty on hemodynamics and muscle histology, this surgical method was evaluated in goats. BACKGROUND: Dynamic cardiomyoplasty has been introduced as a new method to treat patients with severe cardiac failure. METHODS: In 24 goats, the left latissimus dorsi muscle was wrapped around the heart. The muscle was then subjected to progressive electrical stimulation. In 16 goats, invasive transesophageal Doppler echocardiographic measurements and histologic evaluation of the latissimus dorsi muscle were performed at > or = 12 weeks after the wrapping. RESULTS: Only two goats showed an increase in aortic and left and right ventricular pressures concomitant with increased aortic flow during latissimus dorsi muscle stimulation both before and after induction of cardiac failure using imipramine. This was accompanied by a preserved latissimus dorsi muscle structure and nearly complete transformation to type I muscle fibers. The remaining 14 goats showed extensive lipomatosis in the latissimus dorsi muscle, with severe intimal hyperplasia and proliferation of smooth muscle cells in the walls of the thoracodorsal artery and its branches. An increase in endoneural and endomysial connective tissue was observed, with some goats showing destroyed nerve branches near the electrodes. These findings differed from those observed after long-term electrical stimulation of goat latissimus dorsi muscle in situ. CONCLUSIONS: Dynamic cardiomyoplasty is of use in the treatment of severe heart failure if the histologic structure of the wrapped latissimus dorsi muscle remains intact. Long-term results in goats suggest that the current approach used in dynamic cardiomyoplasty may lead to deterioration of the wrapped muscle.

Animals↗

Diastematomyelia in adults. Modern imaging and operative treatment.

Diastematomyelia, an uncommon dysraphic state usually seen in children, is a rarity in adults; only 19 reports have been published in the literature so far. Two cases of diastematomyelia in adults are presented and the relevant literature is reviewed. Both of these were investigated by computed tomography and magnetic resonance imaging. These two procedures are proved to be complementary to each other in understanding this anomaly. Both patients exhibited associated congenital abnormalities of the neuroectoderm in the form of hyperpigmentation, hypertrichosis, lipomatosis, and dermal sinus that were demonstrated with clarity by the neuroimaging modalities used. Operative correlation was performed in both patients, where findings correlated very well with the preoperative radiological evaluation.

Adult↗

Clinical and histopathological findings in Bannayan-Riley-Ruvalcaba syndrome.

Bannayan-Riley-Ruvalcaba syndrome is a rare autosomal dominant genodermatosis with the classical triad of macrocephaly, genital lentiginosis, and intestinal polyposis. Characteristic mucocutaneous manifestations include vascular malformations, lipomatosis, speckled lentiginosis of the penis or vulva, facial verrucae-like or acanthosis nigricans-like lesions, and multiple acrochordons of the neck, axilla, and groin. We present a case of Bannayan-Riley-Ruvalcaba syndrome with macrocephaly, abnormal facies, lipoma, tender and painful arteriovenous hemangiomas, lymphangiokeratomas, musculoskeletal abnormalities, and localized myopathy. We also describe previously unreported findings, including peripheral neuropathy, punctate cystic changes in acral tubular bones, and enostosis of talus. Bannayan-Riley-Ruvalcaba syndrome needs recognition by dermatologists because affected patients may present with mucocutaneous and subcutaneous lesions that may simulate other dermatological disorders.

Abnormalities, Multiple↗

Lipoma arborescens of the knee.

Lipoma arborescens (diffuse articular lipomatosis) is a rare, benign intra-articular lesion of unknown aetiology. It is characterised by villous proliferation of the synovium and diffuse replacement of the subsynovial tissue by mature fat cells. It forms part of the differential diagnosis for a slowly progressive chronically swollen knee. We present a very rare case of bilateral involvement of the knee and discuss the symptoms, diagnosis and treatment of this condition.

Adult↗

Altered expression of master regulatory genes of adipogenesis in lipomas from patients bearing tRNA(Lys) point mutations in mitochondrial DNA.

The mechanisms underlying the appearance of lipomas in patients bearing mutations in the tRNA(Lys) gene of mitochondrial DNA are unknown. We investigated changes in gene expression patterns in lipomas from three patients bearing A8344G or G8363A tRNA(Lys) gene mutations. Uncoupling protein-1 mRNA was detected in the lipomas, in contrast with undetectable expression in normal adipose tissue. However, expression of other markers of brown fat, such as PGC-1alpha, was unaltered. PPARgamma and retinoblastoma gene expression was down regulated in the lipomas, but C/EBPalpha mRNA was not affected. The expression of Pref-1 was dramatically down regulated. Thus, lipomatosis due to tRNA(Lys) mutations is associated with a pattern of altered expression of master regulators of adipogenesis consistent with enhanced proliferation but maintenance of adipocyte features, and with a distorted pattern of brown versus white adipocyte differentiation.

Adipogenesis↗

The use of computed tomography to evaluate suspected mediastinal tumors.

Thirty patients with suspected mediastinal tumors were evaluated by computed tomography (CT) at UCLA Medical Center. Twenty patients with myasthenia gravis were examined for possible thymomas, benign and malignant; and 10 patients were studied for other mediastinal masses (including teratoma, seminoma, mediastinal lipomatosis, carcinoma, lymphoma, and paravertebral abscess). The CT scan was found useful in several respects: (1) yielding information not available by conventional radiographic techniques; (2) defining the anatomical location and extent of mediastinal tumors; (3) detecting pulmonary metastasis and involvement of mediastinal nodes in cases of malignancy; and (4) establishing the diagnosis of benign mediastinal fatty masses. On the basis of our early experience, we believe CT is a valuable adjunct in the preoperative assessment of patients with suspected mediastinal tumors.

Humans↗

Cloning and initial characterization of human and mouse Spot 14 genes.

The intricate regulation of Spot 14 expression in rat lipogenic tissues has provided a useful tool in studying nutritional and hormonal factors involved in transcription. To gain insight into its function and its possible involvement in human lipid disorders, we cloned human and mouse Spot 14 genes that shared with the rat gene a strong homology concerning the deduced amino acid sequence (81 and 94%, respectively) as well as the promoter region. The mouse promoter was characterized by transfection studies, while quantitative RT-PCR and in situ hybridization experiments showed that Spot 14 is expressed in human liver and, at a high level, in multiple symmetric lipomatosis nodules.

Amino Acid Sequence↗

Prevesical xanthogranulomatous pseudotumor.

We describe a patient who was referred to our hospital because of dysuria, frequency and a palpable mass in the suprapubic area. A specimen obtained at suprapubic exploration consisted of fatty tissue and tentative diagnosis was pelvic lipomatosis. The symptoms persisted and the entire mass was removed from its prevesical pelvic position. The pathological report was xanthogranuloma. Postoperatively, the symptoms disappeared.

Granuloma↗

Polycystic disease of the renal sinus: structural characteristics.

Based on a series of 32 identified cases the radiologic, sonographic and anatomic features of polycystic disease of the renal sinus are described. This entity is a bilateral accumulation of thin-walled clear fluid-filled cysts, intertwined with the caliceal infundibula and usually confused radiologically with adult polycystic kidney disease or with renal sinus lipomatosis. Computerized tomography and sonography demonstrate the parenchyma to be free of cystic disease and the hilus to be free of fat accumulation. This newly recognized entity has a completely benign natural history.

Humans↗

Bladder wall lipoma.

Bladder wall lipoma is a rare finding that includes mature, well-encapsulated fatty tissue within the submucosa of the bladder wall. We present a case of a submucosal bladder wall lipoma that was discovered incidentally during a workup for microhematuria. This lesion was seen during cystoscopy and removed using cold cup biopsy forceps. This lipoma should be differentiated from pelvic lipomatosis in that it was confined to the submucosa of the bladder wall and did not cause compression of the pelvic viscera or its structures.

Biopsy, Needle↗

Angiomyolipoma of the renal sinus: diagnosis by percutaneous biopsy.

We report a case of angiomyolipoma of the renal sinus discovered incidentally during an evaluation for microscopic hematuria. Diagnosis was confirmed by percutaneous aspiration biopsy performed with magnetic resonance imaging control allowing differentiation of this entity from other fatty tumors of the renal sinus including liposarcoma, lipoma, and sinus lipomatosis.

Angiomyolipoma↗