1/12. Phosphaturic mesenchymal tumor-induced rickets.We describe two prepubertal girls with oncogenic rickets. The first patient, 9 years of age, presented with recent-onset lower-extremity pain. The second girl, presented at 4 years of age following a 9-month period of muscle weakness, bone pain, and poor linear growth. Laboratory analyses in both patients revealed hypophosphatemia and hyperphosphaturia; elevated circulating alkaline phosphatase activity was present in one of them. Radiographic evidence of a generalized rachitic process was evident in both cases. Computerized tomography of the paranasal sinuses and facial bones in patient 1 revealed a small lesion eroding through the inner table of the left mandibular ramus. Microscopic examination of this mass revealed a spindle cell neoplasm with chondroid material, dystrophic calcification, and both osteoclast-like and fibroblast-like cells. Prominent vascularity and marked atypia were present. These features are consistent with a phosphaturic mesenchymal tumor of the mixed connective tissue variant. In the second patient, computerized tomography revealed a lytic lesion located in the right proximal tibia, with histologic features consistent with a phosphaturic mesenchymal tumor of the nonossifying fibroma-like variant. Resection of each tumor resulted in rapid correction of the phosphaturia and healing of the rachitic abnormalities. A careful search for small or occult tumors should be carried out in cases of acquired phosphaturic rickets.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
2/12. Phosphaturic mesenchymal tumor, mixed connective tissue variant (oncogenic osteomalacia).A case of tumor-induced phosphaturic osteomalacia in a 54 year old man is reported. The patient was admitted because of progressive muscle spasms with pain and weakness in the bilateral thighs. Laboratory data showed hypophosphatemia, decreased tubular resorption of phosphate (TRP), a low 1,25-dihydroxyvitamin D level, and a high serum alkaline phosphatase level. Radiologic examinations revealed multiple lesions of osteomalacia in the ribs, and a small mass in the lower posterior mediastinum. After removal of the tumor, clinical symptoms disappeared and hypophosphatemia, decreased TRP, and the 1,25-dihydroxyvitamin D level were corrected. Microscopical examination revealed that the tumor was composed of mature adipose tissues, osseous tissues, and primitive stromal zones including osteoclast-like giant cells, non-mineralized woven bone, and various sized blood vessels. Patho-physiologic observations suggested that the tumor secreted some humoral substances inhibiting 25-hydroxyvitamin D-1 alpha-hydroxylase activity, renal phosphate resorption, and parathyroid hormone production.- - - - - - - - - - ranking = 4keywords = mixed connective, connective (Clic here for more details about this article) |
3/12. A neuroendocrine cause of oncogenic osteomalacia.All definite cases of oncogenic osteomalacia have, until now, been classified as mesenchymal tumours. We report here a case of oncogenic osteomalacia caused by a spinal tumour. Microscopically, it resembled the mixed connective tissue variant of previously described phosphaturic tumours. Immunohistochemical studies, however, showed the tumour cells to be positive for low molecular weight cytokeratin (CAM 5.2), S100 protein, PGP 9.5, and synaptophysin. Electron microscopy demonstrated neurosecretory granules. The histopathological findings strongly suggest that this is a neuroendocrine tumour.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
4/12. August 2003: 47-year-old female with a 7-year history of osteomalacia and hypophosphatemia.The August 2003 COM. A 47-year-old woman presented with a long history of muscle pain, weakness, and visual disturbances. Over the last year, she developed diplopia and left sixth nerve palsy. No other neuro-ophthalmologic abnormalities were found. Past medical and family history was unremarkable. Laboratory investigation disclosed hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase activity, and normal serum calcium levels. CT scans showed a lobulated mass arising on the meningeal surface of the cavernous sinus, measuring 3x 2 x 2 cm. The lesion was partially resected and microscopic examination revealed a highly vascularized tumor composed of primitive mesenchymal cells arranged whether in a patternless-pattern or surrounding thin-walled, branching vascular spaces and thick-walled hyalinized vessels. Other eye-catching features were microcystic areas, multinucleated osteoclastic-like giant cells, scattered islands of mature adipocytes, foci of hemorrhage, thrombosed medium-sized-to-large vessels, and hemosiderin-laden macrophages. After surgery, the patient recovered from the muscle pain and weakness, with a significant improvement of serum calcium and alkaline phosphatase levels and phosphate blood levels. Taken together, the clinical and pathologic features were consistent with a diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue variant) of the cavernous sinus in a setting of oncogenic osteomalacia. No further treatment was carried out. The patient has been followed for the last 4 years with no evidence of recurrent disease. Oncogenic osteomalacia is a rare clinical entity characterized by hypophosphatemia, phosphaturia, and a low concentration of 1,25-dihydroxyvitamin D(3) caused by a neoplasm. Pathologists should be aware of this entity, because surgical excision of the tumor is usually curative.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
5/12. Calcifying panniculitis following subcutaneous injections of nadroparin-calcium in a patient with osteomalacia.Calcifying panniculitis is a rare form of calcinosis cutis belonging to the spectrum of calciphylaxis that has almost invariably been described in patients with severe renal disturbances. We report a patient with osteomalacia without chronic renal failure, who developed calcifying panniculitis following subcutaneous administration of nadroparin-calcium. light microscopy studies of biopsy specimens revealed multiple foci of microcalcification within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the media of small arteries in the subcutis. The patient had an elevated level of intact parathyroid hormone, whereas the calcium-phosphorus product was normal. The lesions slowly resolved upon discontinuation of nadroparin. We conclude that calcifying panniculitis is a rare complication associated with the subcutaneous administration of nadroparin-calcium that may rarely also occur in the absence of renal disturbances. Low molecular weight calcium-containing heparins should probably be used with caution in the presence of hyperparathyroidism.- - - - - - - - - - ranking = 0.025142236637809keywords = connective (Clic here for more details about this article) |
6/12. Phosphaturic mesenchymal tumor: a case report.osteomalacia induced by tumor is a rare phenomenon in which the resection of tumor is followed by dramatic amelioration of clinical signs and symptoms. We hereby report a case of a 66 years old male who presented with features of osteomalacia in which the characteristic clinical presentation was associated with the phosphaturic mesenchymal tumor, mixed connective tissue variant. The case is reported for its rarity.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
7/12. Oncogenic osteomalacia in a case with a maxillary sinus mesenchymal tumor.We herein describe the rare case of a 41-year-old woman with oncogenic osteomalacia due to a tumor in the maxillary sinus who presented with chronic general pain that had been gradually deteriorating. The patient's laboratory findings revealed hypophosphatemia due to renal phosphate wasting, an inappropriately low serum 1 alpha,25-dihydroxyvitamin D3 level for hypophosphatemia and an unusually high serum level of fibroblast growth factor 23 (FGF23). The causative tumor was surgically removed, resulting in a rapid resolution of the patient's biochemical abnormalities. An improvement of the abnormal multiple deposits on (99)technetium-methylene diphosphonate bone scintigraphy and an increase in the bone metabolism markers suggested the development of bone remodeling within 49 days after the operation. The pathologic diagnosis of the tumor was a "phosphaturic mesenchymal tumor, mixed with a connective tissue variant." The expression of FGF23 was demonstrated in the tumor by the immunohistochemical techniques and a Western analysis.- - - - - - - - - - ranking = 0.025142236637809keywords = connective (Clic here for more details about this article) |
8/12. Phosphaturic mesenchymal tumor with symptoms related to osteomalacia that appeared one year after tumorectomy.A 45-year-old man was admitted to our hospital because of bone pain and hypophosphatemia. He had undergone surgery 2 years previously for a "benign unclassified mesenchymal tumor" in the skull, but there were no clinical symptoms related to osteomalacia. His laboratory examination revealed low serum phosphate, high alkaline phosphatase, and normal calcium levels. The diagnosis of tumor-induced osteomalacia due to phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) was made by re-examining the pathologic specimens. Oral supplementation with phosphate and 1-25-dihydroxyvitamin D relieved his clinical symptoms and laboratory values returned to normal. However, subcutaneous administration of octreotide had no clinical effect. Clinicians and pathologists should be aware of the existence of PMTMCT especially nonphosphaturic or asymptomatic variants of this disorder.- - - - - - - - - - ranking = 1keywords = mixed connective, connective (Clic here for more details about this article) |
9/12. Hypophosphatemic oncogenic osteomalacia: report of three new cases.Three cases of connective tissue tumors causing hypophosphatemic osteomalacia are reported and the literature is reviewed. In two of our patients the tumors were completely excised with total disappearance of the symptoms. In one case a total excision was not possible and the symptoms of this patient have not completely disappeared. The substance responsible for the syndrome has not been identified yet, but probably interferes with vitamin d renal hydroxylation, thus causing osteomalacia. As more than 30 per cent of cases of this condition have been reported in the last 5 years, it is suggested that these tumors are more frequent than previously believed.- - - - - - - - - - ranking = 0.025142236637809keywords = connective (Clic here for more details about this article) |
10/12. osteomalacia as a presenting manifestation of sjogren's syndrome.osteomalacia is still common in morocco, where the leading causes are nutritional deficiencies followed by intestinal diseases. osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to a connective tissue disease such as sjogren's syndrome. The case of a 40-year-old woman who presented with a five-year history of generalized bone pain, severe weight loss and a waddling gait is reported. She had low levels of serum phosphate (0.74 mmol/L), serum calcium (1.97 mmol/L), and urinary calcium (1.22 mmol/24 h). serum alkaline phosphatase was 210 IU/L. Roentgenograms showed Looser's zones (right femoral neck, sixth and seventh right ribs). There was bilateral parotid gland enlargement, dryness of the mouth, nose and eyes, and bilateral punctate keratitis. A lip biopsy showed changes corresponding to stage II of the Chisholm and Mason classification. Tests for rheumatoid factor (latex and Waaler-Rose) and antinuclear factor were negative. The alkaline reserve was 18 mmol/L, serum potassium was 3.5 mmol/L, serum chloride was 112 mmol/L and urinary pH was 6.5. A renal biopsy showed tubulointerstitial lesions, lymphoplasmocytic infiltrates and interstitial sclerosis with patchy tubular atrophy. The patient was given bicarbonates, high-dose vitamin d followed by 1-alpha-hydroxycholecalciferol (0.3 microgram/d), and calcium (1 g/d). Follow-up was 42 months at the time of this writing. The role of tubular disorders in the genesis of osteomalacia is discussed, and the renal manifestations of sjogren's syndrome are reviewed.- - - - - - - - - - ranking = 0.025142236637809keywords = connective (Clic here for more details about this article) |
| | Next -> |