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Oncogenous Osteomalacia - Report of a Case

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  Actci Oncolo,qic.ri Vol. 33. No. 8, pp. 975-980. 1994 Correspondence and ShortCommunications Cotiinwnts on published urticlt~, hort c.ommunicatioris (?/ 11 prelinzinnr~~ uturc. c'use reports, teclinictil no/es and rhc like tire uccepttd iintkr this lieuding. The cirticltJs should be si~ort imi concise rmrl contuin CI minimum of jgures. trrbles rind rqfi~rcwc~s. ONCOGENOUS OSTEOMALACIA-REPORT OF A CASE Oncogenous osteomalacia is an unusual clinical syndrome char-acterized by a solitary or focal soft tissue and/or bone tumorproducing osteomalacia ( 1) and manifested by bone pain.. patho-logic fractures, marked hypophosphatemia, hyperphosphaturia.normal to low level of serum calcium, normal levels of parathy-roid hormone and elevated serum alkaline phosphatase (3. 3).Serum 1.25-dihydroxyvitamin D, was found to be low in nearlyall cases (4). Diffuse osteoporosis and/or Loozer's zones may beseen in all bones. The tumors associated with oncogenous os-teomalacia are usually benign and characterized by the predonii-nance of giant cells and spindle cells (87'%,) nd high degree ofvascularity (XO'X)) (I. 5). Complete excision of tumors results incure in the majority of patients (6). Cmr repor/. A 46-year-old white man was admitted to DokuzEylul University Hospital for increasing pains localized to righthip joint, right femur, sternum. and ribs and progressive weaknesscausing marked difficulty in walking. He was first examined in another hospital in June 1989 because of similar pains with 3years' duration. x-Rays of the right femur then showed lytic bonelesions in the right intertrochanteric area. CT of pelvis revealed ;I tumor mass in the collum of right femur eroding the cortex of thebone and invading into the surrounding soft tissues. The patientunderwent two curettages of this lesion and bone grafting in J tineand September 1989. Histopathologic examination showed osteo-chondroblastonia. Physical examination on admission to our hos-pital revealed marked limitation of movement in the right hipjoint. tenderness in this joint. sternum and chest wall. His bloodchemistry profile was within normal limits except for elevatedalkaline phosphatase 273 U/L (normal: 35- 123). decrease in inor-ganic phosphorus I I mg/l (normal: 25-48). His serum calciumwas normal. 101 mg/l (normal: 88- I1 I). x-Rays of spine. pelbisand long bones revealed diffuse osteoporosis and a lytic lesion in the right intertrochanteric area. MRI of pelvis and right femurshowed a tumor mass in the right intertrochanteric area withcortical and medullary damage and protrusion into the surround-ing soft tissues (Fig. I). Radionuclide bone scanning showedincreased uptake in vertebrae. ribs and pelvis. A posterior segmentof the right 7th rib. showing increased radionuclide uptake, wasbiopsied and histopathology revealed callus formation. vasculiirproliferation and osteomalacia but no atypical cells (Fig. 2).Review of slides prepared from paraffin blocks of the tumor in theright intertrochanteric area in June 1989 showed tumor tissuecomposed of round. ovaloid and/or spindle shape cells and promi-nent vascularity with reticular strorna. Osteoclast-like giant cellswere also identified in the areas of tumor tissue. Unmineralixedosteoid tissue which is typically seen in osteomalacia was foundaround the lesion. Biochemical studies on 24-h urine demon-strated glycosuria. proteinuria and hyperphosphaturia withoutaminoaciduria. Blood chemistry showed decreased inorganicphosphorus of 16 mg/l (normal: 25-48). His fasting blood glucosewas 980mg/l (normal: 700-1100), blood pH 7.42 and bloodbicarbonate 27 mmol/l (normal: 23-26). The serum parathyroidhormone level was normal. Parathyroid scintigraphy failed to Fig. I. Demonstrating a tumor mass localized in the right in-tertrochanteric area producing cortical and medullary damagewith protrusion into the surrounding soft tissues by MRI. Ftg. 2. Biopsy from a rib showing osteoid tissue with thin walled-large dilated vessels filled with red blood cells. small capillaryvessels and osteoclast-like rnultinuclcated giant cells (HE x 400). demonstrate adenoma or hyperplasia. Serum 25-hydroxycholecal-ciferol and 1.25-dihydroxycholecalciferol evels could not be de-termined. The diagnosis of oncogenous osteomalacia wasestablished and in February 1992 the tumor in the one thirdproximal part of right femur was surgically removed and re-placed by an artificial hip joint. Following the surgery his symp-toms cleared up in about 2 weeks. Thc blood inorganicphosphorus returned to nornial level one month later with thedisappearance of hyperphosphaturia in the urine His last visitwas in December 1993 and he has been doing very well withoutany evidence of disease. DiscriJsion. In the reported case the diagnosis of oncogenousosteomalacia was made by detection of hypophosphatemia, andhyperphosphaturia with normal levels of serum calcium andparathyroid hormone and demonstration of vascular proliferationand osteomalacia in the resected rib in addition to the findings of 91 5    A  c   t  a   O  n  c  o   l   D  o  w  n   l  o  a   d  e   d   f  r  o  m    i  n   f  o  r  m  a   h  e  a   l   t   h  c  a  r  e .  c  o  m    b  y   1   1   7 .   1   9   3 .   2   3   2 .   3   F  o  r  p  e  r  s  o  n  a   l  u  s  e  o  n   l  y .  976 CORRESPONDENCE Acta Oncologica Vol. 33, No. 8, pp. 975-980, 1994spindle shape and osteoclast-like giant cells and prominent vascu-larity in the excised tumor by histopathologic examination anddiffuse osteoporosis by x-rays. Disappearance of hypophos-phatemia, hyperphosphaturia, glycosuria, proteinuria and the dra-matic clearance of his symptoms following removal of the tumorwere also con-firmatory evidences for the diagnosis of oncogenousosteomalacia. Recognition and removal of the tumor in patientswith oncogenous osteomalacia restores the severe and total dis-ability (7). In conclusion, oncogenous osteomalacia should besuspected in any patient who presents with metabolic bone diseaseassociated with hypophosphatemia and inappropriate phospha-turia, in combination with a benign mesenchymal soft tissue or bone tumor. The possibility of metastatic malignant diseaseshould be ruled out by biopsies of the bones guided by bone scan or x-ray examinations.CAVIT EHRELI’MEHMET . ALAKAVUKLAR’CANER AVDAR* BULENT UNDAR’NURULLAHKKOC’BAHRIYE AYZIN‘GULCIN BASDEMIR’ FIKRI ZTOP’ Division of Hematology & Oncology’ andDepartment of InternalMedicine’Dokuz Eylul University,School of Medicine.and Department of Pathology3Ege University,School of MedicineIzmirTurkeyApril 1994Correspondence to: Dr Cavit Cehreli, Division of Hematology & Oncology, Dokuz Eylul University, School of Medicine, 35340,Inciralti, Izmir, Turkey. I. 3 -. 3.4. 5. 6.7. REFERENCES Edmond AR, Reiss E. Oncogenic osteomalacia. Am J Med1984; 77: 501-12.Agus ZA. Oncogenic hypophosphatemic osteomalacia. KidneyInt 1983; 24: 113.Wener M, Cohen L, Bar RS, Strottmann MP, DeLuca H.Regulation of phosphate and calcium metabolism by vitamin Dmetabolites: Studies in a patient with oncogenic osteomalacia.Arthritis Rheum 1979; 22: 672-3.Sweet R, Males J, Hamstra A, Deluca H. Vitamin D metabo-lite levels in oncogenic osteomalacia. Ann Intern Med 1980; 93:279-80.Weidner N, Bar RS, Weiss D, Strottmann MP. Neoplasticpathology of oncogenic osteomalacia/rickets. Cancer 1985; 55:1691 -705.McGuire HM, Meranda J, Etzkorn J, Sundaram M. Oncogenicosteomalacia. Clin Orthop Relat Res 1989; 244: 305-8.Cotton GE, Puffelen P. Hypophosphatemic osteomalacia sec-ondary to neoplasia. J Bone Joint Surg 1986; 68-A: 129-33. IS GASTRIC CANCER HETEROGENEITY THECLUE TO HLA-DR ASSOCIATED SUSCEPTIBILITY? Worldwide, gastric cancer remains a leading cause of cancerdeath (I). Despite recent advances (2), the role of genetic andimmune factors for the development of gastric cancer remainsunclear. A genetic component was suggested when increasedfrequency of blood group A was found in patients with gastriccancer (3). Subsequent studies have confirmed that heredity maybe important for the development of this malignancy (4-7). Thegenetic regulation of immune phenomena mainly occurs withinMHC, and HLA-DR antigens play a crucial part in initiating thespecific immune response. It has therefore been suggested thatphenotypic HLA-DR differences may be associated with differentrisks of developing neoplastic diseases. However, in three reportedpopulation studies on HLA-DR and gastric cancer no constantrelation was found (8- lo), and the same concerned earlier investi-gations of HLA class I antigens (11-13). One reason for theconflicting results may be gastric cancer heterogeneity (I, 2, 14,15). These facts prompted us to investigate the association of HLA-DR antigens with gastric cancer taking into considerationits types according to the Jarvi-Lauren classification, tumourgrade, stage, and topographical classification. Material and Methods. Forty-six consecutive, unrelated patients(35 males and 11 females) of Polish ancestry with resectable andhistologically proven gastric carcinoma have been prospectivelytested since 1989 and typed for 10 HLA-DR specificies (DRI,DR2, DR3, DR4, DR5, DRw6, DR7, DRw8, DR9, DRwlO)by a standard microlymphocytotoxicity technique (16, 17).Lymphocytes were isolated by gradient centrifugation and T andB cell separation was performed with AET-treated sheep erythro-cytes. The serotyping trays were ‘Lymphotype-DR’ (Biotest Diag-nostics). The controls consisted of 389 Polish individuals. Antigenfrequencies were calculated in the control group, the total cancergroup and in subgroups of patients categorized according to theJarvi-Lauren classification ( 14) (24 patients with intestinal type(Int), 14 with diffuse type (Dif and 32 with intestinal and mixedtypes (Int + M)), WHO histological grading (15) (26 patients withadenocarcinomas with high or moderate grade of differentiation(H + M) and 20 with poorly differentiated adenocarcinoma or undifferentiated carcinoma (P + U)), TNM-AJC classification oftumour extension-T (15) (12 patients with TI or T2,43 with T2 or T3 or T4, 22 with TI or T2 or T3, and 24 with T4) andtopographical classification ( 15 patients with the tumour locatedin the cardiac region-Car, 27 in the fundus or body-Fun + Cor, 21 in the pyloric region-Pyl). The antigen frequen-cies in the groups were compared using the x2 test with Yates’correction. The strength of association was estimated by calculat-ing the relative risk (RR). Results. HLA-DR typing results in the 46 patients with gastriccancer are shown in Table I. Only the HLA-DR5 frequency was Table 1 HLA -DR antigens distribution among 46 gastric cancer patientsand 389 controls HLA Frequency (YO) requency (YO) z p-valuespecificity patients controlsDR 1 DR2DR3DR4DR5*DRw6DR7DRw8DR9DRwlODRx28.323.919.617.434.813.021.74.34.34.328.320.325.222.419.320.114.926.55.73.63.338.6 * RR = 2.13DRx- unidentified antigens or homozygotes1.12 <O.Ol 0.06 0.01 4.44 0.01 0.27 < 0.0 0.03 <O.Ol 1.45N.S.N.S.N.S. N.S. < 0.05N.S.N.S.N.S.N.S.N.S.N.S.    A  c   t  a   O  n  c  o   l   D  o  w  n   l  o  a   d  e   d   f  r  o  m    i  n   f  o  r  m  a   h  e  a   l   t   h  c  a  r  e .  c  o  m    b  y   1   1   7 .   1   9   3 .   2   3   2 .   3   F  o  r  p  e  r  s  o  n  a   l  u  s  e  o  n   l  y .

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