Clinical Endocrinology (1992)37,379-384
Case of the Month
Oncogenous osteomalacia and malignancy J. N. Harvey, C. Gray' and P. E. Belchetz Department of Endocrinofogy, General Infirmary at Leeds, Leeds LS13EX and "Department of Pathology, University of Leeds, Leeds LS2 9JT, UK
nant neoplasm. This case is compared with the eight previously described cases of oncogenous osteomalacia in association with malignant tumours.
(Received 4 February 1992; returned for revision 28 February 1992; finally revised 17 March 1992; accepted 8 April 1992)
Case report
Summary An example of oncogenous osteomalacia Is described In asroclationwith the rare mesenchymalchondroblastoma. The tumour was characterlzedwith a varlety of lmmunohlstochemlcal antlbodles which conflrmed I t s mesenchyma1 orlgln. Despite several surgical excislons of local recurrence, plasma 1,25-dlhydroxyvitamln Ds concentrations (off treatment) indlcate continuedtumour activity and the contlnulng need for vltamln D therapy. This case and two others brlefly discussed Illustratesome of the dlfflcultles in dlagnosis and In achleving a cure In cases of oncogenous osteomalacla. Oncogenous osteomalacla in association with any malignant tumour is rare, there being eight previously recorded cases. Details of these cases are briefly revlewed for comparison. Despite undoubted mallgnancy our patient has survived 30 years from presentatlon indlcatlng the benefits of aggressive surgical management.
Oncogenous osteomalacia is an uncommon syndrome in which neoplasms usually of a mesenchymal nature are found in association with non-familial adult-onset osteomalacia. These tumours appear to elaborate an as yet unidentified humoral factor which is responsible. Some 50 examples are documented in the literature and are reviewed elsewhere (Ryan & Reiss, 1984; Segre & Dickersin, 1989). The majority of tumours were benign. We here describe an example associated with mesenchymal chondrosarcoma, a rare malig-
Correspondence: Dr J. N. Harvey, c/o Dr Barnett's Secretary, Chancellor Wing, St James's Hospital, Beckett Wing, Leeds LS9 TTF, UK. *Current address: Department of Histopathology, Harrogate General Hospital, Harrogate HG2 7ND, UK.
In 1962 a female patient aged 32 developed generalized discomfort thought subsequently to be bone pain. Two years later she developed a mass in the neck and underwent hemithyroidectomy and block dissection for a neoplasm thought at operation to be arising from the thyroid. Her aches and pains subsided but had returned by 1970when she suffered fractures of femur and pubic ramus. Recovery was complicated by pulmonary embolism. Osteomalacia was diagnosed in 1974 when she had become weak to the point of walking difficulty with myopathic involvement of shoulder and hip girdle muscles. Her plasma calcium was 2.4 mmol/l, phosphate 0.56 mmol/l and alkaline phosphatase 21 KingArmstrong units (KAU). X-rays showed numerous Looser's zones in scapulae, ribs and pelvis. Investigation excluded malabsorption, thyrotoxicosis, aminoaciduria and systemic acidosis. Radiocalcium absorption and excretion were low. Muscle biopsy showed non-specific myopathic changes. She was treated with alfacalcidol, initially 6 pg daily with phosphate, subsequently reduced to 2 pg daily alone. In 1975 she collapsed at home then suffered a tonic-clonic convulsion on the ward. Plasma calcium was 2.9 mmol/l and phosphate 0.86 mmol/l immediately afterwards. In 1980 she was readmitted following 2 tonic-clonic convulsions and started on sodium valproate. Recurrence of the tumour was noted and she underwent a partial laryngectomy. Alfacalcidol was stopped in 1981 because she was felt to be cured but in 1984 a total laryngectomy followed by radiotherapy was necessary. Three years later symptomatic and biochemical osteomalacia recurred with new Looser's zones. Plasma FTH was 0-38 pg/l (normal
Case of the Month
Oncogenous osteomalacia and malignancy J. N. Harvey, C. Gray' and P. E. Belchetz Department of Endocrinofogy, General Infirmary at Leeds, Leeds LS13EX and "Department of Pathology, University of Leeds, Leeds LS2 9JT, UK
nant neoplasm. This case is compared with the eight previously described cases of oncogenous osteomalacia in association with malignant tumours.
(Received 4 February 1992; returned for revision 28 February 1992; finally revised 17 March 1992; accepted 8 April 1992)
Case report
Summary An example of oncogenous osteomalacia Is described In asroclationwith the rare mesenchymalchondroblastoma. The tumour was characterlzedwith a varlety of lmmunohlstochemlcal antlbodles which conflrmed I t s mesenchyma1 orlgln. Despite several surgical excislons of local recurrence, plasma 1,25-dlhydroxyvitamln Ds concentrations (off treatment) indlcate continuedtumour activity and the contlnulng need for vltamln D therapy. This case and two others brlefly discussed Illustratesome of the dlfflcultles in dlagnosis and In achleving a cure In cases of oncogenous osteomalacla. Oncogenous osteomalacla in association with any malignant tumour is rare, there being eight previously recorded cases. Details of these cases are briefly revlewed for comparison. Despite undoubted mallgnancy our patient has survived 30 years from presentatlon indlcatlng the benefits of aggressive surgical management.
Oncogenous osteomalacia is an uncommon syndrome in which neoplasms usually of a mesenchymal nature are found in association with non-familial adult-onset osteomalacia. These tumours appear to elaborate an as yet unidentified humoral factor which is responsible. Some 50 examples are documented in the literature and are reviewed elsewhere (Ryan & Reiss, 1984; Segre & Dickersin, 1989). The majority of tumours were benign. We here describe an example associated with mesenchymal chondrosarcoma, a rare malig-
Correspondence: Dr J. N. Harvey, c/o Dr Barnett's Secretary, Chancellor Wing, St James's Hospital, Beckett Wing, Leeds LS9 TTF, UK. *Current address: Department of Histopathology, Harrogate General Hospital, Harrogate HG2 7ND, UK.
In 1962 a female patient aged 32 developed generalized discomfort thought subsequently to be bone pain. Two years later she developed a mass in the neck and underwent hemithyroidectomy and block dissection for a neoplasm thought at operation to be arising from the thyroid. Her aches and pains subsided but had returned by 1970when she suffered fractures of femur and pubic ramus. Recovery was complicated by pulmonary embolism. Osteomalacia was diagnosed in 1974 when she had become weak to the point of walking difficulty with myopathic involvement of shoulder and hip girdle muscles. Her plasma calcium was 2.4 mmol/l, phosphate 0.56 mmol/l and alkaline phosphatase 21 KingArmstrong units (KAU). X-rays showed numerous Looser's zones in scapulae, ribs and pelvis. Investigation excluded malabsorption, thyrotoxicosis, aminoaciduria and systemic acidosis. Radiocalcium absorption and excretion were low. Muscle biopsy showed non-specific myopathic changes. She was treated with alfacalcidol, initially 6 pg daily with phosphate, subsequently reduced to 2 pg daily alone. In 1975 she collapsed at home then suffered a tonic-clonic convulsion on the ward. Plasma calcium was 2.9 mmol/l and phosphate 0.86 mmol/l immediately afterwards. In 1980 she was readmitted following 2 tonic-clonic convulsions and started on sodium valproate. Recurrence of the tumour was noted and she underwent a partial laryngectomy. Alfacalcidol was stopped in 1981 because she was felt to be cured but in 1984 a total laryngectomy followed by radiotherapy was necessary. Three years later symptomatic and biochemical osteomalacia recurred with new Looser's zones. Plasma FTH was 0-38 pg/l (normal
