An adult form of Gaucher's disease with a huge tumour formation of the right tibia

Summary A case of adult, chronic or non-neuropathic, Gaucher's disease is presented. Severe bony changes, particularly involving the lower limbs developed after a splenectomy had been carried out. Our patient had all the orthopaedic complications of Gaucher's disease. Further, a huge tumour was present in the right tibia caused by the proliferation of Gaucher cells. This feature may be unique but pseudotumour should be listed in the possible orthopaedic complications of Gaucher's disease. The patient was bedridden, but was able to use a wheelchair after his leg was amputated.

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Résumé Présentation d'un adulte atteint de maladie de Gaucher dans sa forme chronique, non neuropathique. De graves lésions osseuses, atteignant notamment les membres inférieurs sont apparues après splénectomie. Ce patient présentait toutes les complications orthopédiques de la maladie de Gaucher. En outre, il existait au niveau du tibia droit une énorme tumeur résultant de la prolifération des cellules de Gaucher. Cette observation est peut être unique mais on doit cependant savoir que les pseudo-tumeurs sont une des complications orthopédiques possible de la maladie de Gaucher. Le malade était grabataire, mais il a pu se déplacer à l'aide d'un fauteuil roulant après qu'il ait été amputé de sa jambe.

     

Chondroid lipoma: MRI appearances with clinical and histologic correlation

 Chondroid lipoma is a recently described benign tumor of adipose origin which, pathologically, mimics myxoid liposarcoma and myxoid chondrosarcoma. This tumor typically presents as a well-defined mass in the superficial tissues of the extremities. In this report, we present the magnetic resonance imaging (MRI) appearances of a pathologically proven chondroid lipoma. To our knowledge, this is the first report of any imaging findings associated with this tumor.     

Intraosseous Lipoma: A Report of Two Cases

Intraosseous lipoma seems to be a rare condition as only some 20 cases have been reported so far. A further two cases are described the lesions being in the calcaneus and in the tibia. Radiographically the lesions appeared osteolytic and well delineated, containing calcified areas. The microscopic features were those of mature adipose tissue. Curettage and packing with autogenous bone grafts is recommended, if the lesion causes pain or a correct diagnosis cannot otherwise be obtained.     

Studies on the intraosseous blood circulation and the bone marrow pressure in human lumbar vertebrae

Summary Studies on the bone blood circulation of the 4th lumbar vertebra in patients with or without spondylosis were carried out using the method of radiation clearance study, intravertebral blood gas analysis, intravertebral pressure measurements and osteovenography.Decrease of the intravertebral blood circulation within the spondylotic vertebra was demonstrated, and the lesion of the arterial in-flow system was considered as a responsible factor for spondylotic changes rather than the disturbance of venous out-flow system.The pathogenesis of spondylosis is discussed in this study.

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Résumé L'étude de la circulation sanguine au niveau de la 4ème vertèbre lombaire a été réalisée chez des sujets atteints ou non de spondylose rhizomélique, par mesure de la clearance isotopique, par analyse des gaz du sang intravertébral, par mesure de la pression intravertébrale et par ostéoveinographie.La diminution de la circulation sanguine dans la vertèbre spondylosique a été démontrée et la perturbation du système afférent artériel peut être considérée comme le facteur principal plutôt que celle du système veineux efférent.La pathogénie de la maladie est ensuite discutée.

     

Value of cytological imprints in the diagnosis of atypical lipomatous tumor

The diagnosis of atypical lipomatous tumor by biopsy, requires careful examination of several slides, in order to look for atypical cells. We suggest that imprints of lipomatous tumors may play a complementary role in such a search, and report a case of a 74-yr-old woman with an atypical lipomatous tumor of the left thigh, in which imprints helped to make the diagnosis..     

双膝关节滑膜脂肪瘤2例的诊治并文献复习

目的 通过报道2例滑膜脂肪瘤的诊断和治疗,避免临床误诊和误治.方法 报道2例滑膜脂肪瘤患者的临床诊治过程,通过文献回顾,总结该病的临床症状、诊断和治疗方法.结果 滑膜脂肪瘤临床表现无特异性,确诊依靠滑膜病理,治疗的主要手段是滑膜切除术.结论 滑膜脂肪瘤是非常罕见的病因不明疾病,常表现为关节肿胀和反复积液,实验室检查无特异性,膝关节为好发关节,易误诊为骨关节炎、脊柱关节炎等常见病.     

Chondroid lipoma of the right thigh: Correlation of imaging findings and histopathology of an unusual benign lesion

Chondroid lipoma is a rare and unusual benign soft tissue lesion. Although there have been several reports of the imaging findings of chondroid lipoma, very few described the radiographic calcification or ossification in a chondroid lipoma. We present a case of chondroid lipoma with adipose tissue and ossified components on plain radiography and MRI images. Correlation of imaging findings and pathological examination is provided to confirm the diagnosis.     

Diagnostic value and potential clinical significance of duodenal lipoma based on computed tomography imaging data

Background:To explore the diagnostic value of computed tomography (CT) imaging for duodenal lipoma and the potential clinical significance of the findings.Methods:Clinicopathological and CT data from 57 patients, who were diagnosed with duodenal lipoma at the first affiliated Hospital of Zhengzhou University (Zhengzhou, China) between June 2014 and March 2019, were retrospectively reviewed. Data collected included location and size of the tumor, morphological manifestations (shape, density, boundary), concomitant diseases, pathology and gastroscopy results, and follow-up. Follow-up was performed via telephone, and surgical patients were followed-up for recurrence, metastasis and tumor size, and morphological changes. The follow-up period was up to January 2019.Results:Of the 57 patients with duodenal lipoma, contrast-enhanced scanning was performed in 7 cases. The tumor was located in the descending duodenum in 33 cases, the ascending in 4 cases, the horizontal in 16 cases, and the bulb in 4 cases. Mean tumor size was 13.0 ± 5.8 mm. CT morphological features of the tumor were as follows: tumor shape, round, quasi-round, or oval (n = 42); long strip (n = 3); nodular (n = 2); triangular (n = 1); and irregular lobulated (n = 9). Among the 57 patients, tumor density was homogeneous in 52 cases, inhomogeneous in 4 cases, and nodular with calcification in 1 case. The tumor boundary was classified as clear and with no capsule. Diseases concomitant with the tumor were as follows: gastritis (n = 23), gastric adenocarcinoma (n = 1), and gastric lymphoma (n = 1). Esophageal disease was found in 16 cases, including reflux esophagitis (n = 12) and esophageal cancer (n = 4). There were 13 cases of gallbladder and biliary disease, including cholecystolithiasis and cholecystitis (n = 9), common bile duct disease (n = 2), colorectal cancer (n = 4), lung cancer (n = 2), duodenal carcinoma with obstruction (n = 1), and ureteral space narrowing (n = 1).Conclusion:CT was an effective, non-invasive method for diagnosis of duodenal lipoma. CT imaging could clearly discern location, size, shape, and nature of duodenal lipomas. Duodenal lipoma can be associated with digestive tract inflammatory diseases and tumors in different locations, and its diagnosis is potentially valuable for their prevention and treatment.     

Extramammary myofibroblastoma is genetically related to spindle cell lipoma

Extramammary-type myofibroblastoma is a rare, benign spindle cell lesion, strictly resembling the breast counterpart, but occurring in extramammary sites, mainly in the inguinal/groin area. In this paper, we describe an extramammary-type myofibroblastoma in the groin of a 37-year-old male patient. The tumor showed a typical morphological and immunophenotypical profile, including staining for both CD34 and desmin. Dual-color interphase florescent in situ hybridization analysis revealed losses of RB/13q14 and FKHR/13q14 loci within tumor cells. The chromosome 13 rearrangements associated with the loss of the 13q14 chromosomal region are typically seen in spindle cell lipoma, and have been previously recognized in mammary myofibroblastoma, providing strong evidence for a pathogenetic link between these lesions.     

Intracranial lipomas--a clinical study

OBJECTIVE: This study aimed to investigate the localization of the lipoma, as well as associated intracranial and extracranial lesions in 14 patients immediately following hospital admission. The pathological findings from the neurological examinations of these patients are also investigated. METHODS: Fourteen patients who were admitted to our clinic with a variety of symptoms and diagnosed with intracranial lipoma were included in the study. Problems presented upon admission, neurological findings, and other existing system abnormalities were evaluated. Localization of the lipomas and accompanying pathologies were determined by using computerised tomography and magnetic resonance imaging. RESULTS: The most frequent reasons for admission of patients with intracranial lipomas were: headache 7 (50%), trauma 3 (21.5%), epilepsy 3 (21.5%) and one with symptoms due to the local mass effect of tumor (7%). Although the pericallosal region is accepted as the region where lipomas commonly occur, this study found the most frequent occurrence in the quadrigeminal cistern. Intracranial lipoma calcification was only evident in 1 of the 14 patients. In addition, contrary to the expectations, intracranial and extracranial lesions accompanying lipomas were rare. All patients received systematic treatment. CONCLUSION: This study showed that intracranial lipomas are more frequent in the quadrigeminal region of the brain; most are asymptomatic, generally caught incidentally; and accompanying intracranial and extracranial pathologies are less common than expected.