Perioperative concerns in patients with tumor-induced osteomalacia for surgical excision of tumor (original) (raw)
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Anesthesia for oncogenic osteomalacia—A rare paraneoplastic syndrome
Acta Anaesthesiologica Taiwanica, 2012
Two patients with a diagnosis of oncogenic osteomalacia are described. This rare disease, characterized by secretion of fibroblast growth factor-23 by the tumor cells, causes myopathy, extreme debilitation and severe osteopathy because of severe hypophosphatemia. Both patients presented with severe bone pain, pathological fractures and proximal muscle weakness. Multiple diagnostic tools had to be utilized to settle the diagnosis of this rare disease. Although supplemental therapy for hypophosphatemia is usually started preoperatively, surgical excision of the causative tumor is the only definite treatment. Surgery is almost always curative; however, there is a lack of discourse in the literature regarding the anesthetic implications for the disease. The complete pathophysiology of the disease, clinical picture, its diagnostic intricacies as well as the salient points in its anesthetic management are discussed in this report.
Indian Journal of Anaesthesia
Oncogenic osteomalacia (OOM) is a rare paraneoplastic syndrome associated with mesenchymal tumours. It is characterised by phosphaturia, hypophosphataemia, decreased serum Vitamin D3 levels and severe osteomalacia. OOM-inducing tumours are usually benign, arising either from bone or soft tissue, with extremities and craniofacial region being the most common sites. Surgical resection of the tumour remains the mainstay of treatment. Challenges to an anaesthesiologist arise when such patients are planned for surgical resection of the underlying tumour. All the perioperative dilemmas are directly related to the severe hypophosphataemia. We describe three such cases of OOM and their perioperative management.
Current Oncology, 2009
Oncogenic osteomalacia is a rare metabolic bone disease characterized by phosphate leakage from the kidney and subsequent hypophosphatemia. It is caused by a phosphaturic factor produced by certain tumours. Removal of such tumours can completely cure the condition. Here, we report the case of a patient who was crippled with oncogenic osteomalacia. Extensive study revealed a tumour deeply located in the pelvis; removal of the tumour resulted in complete recovery. The tumour was identified as a mesenchymal tumour (mixed connective-tissue variant). The diagnostic evaluation, differential diagnosis, and treatment are discussed.
Tumor-Induced Osteomalacia: A Case Report of Rare Disease and Literature review
South Asian Journal of Cancer
Background Oncogenic osteomalacia term used for tumor-induced osteomalacia (TIO) is a paraneoplastic syndrome of abnormal phosphate metabolism secondary to ectopic endocrine tumors. The diagnosis often becomes difficult due to rarity of occurrence and deficient literature. The reconstruction following resection has its own technical difficulties, which are addressed in this article. Presentation of Case A 39-year-old female presented with pain in bilateral lower limbs and difficulty in mobilizing. The patient had unexplained hypophosphatemia which was diagnosed due to tumor (arising ectopically in greater trochanter), inducing osteomalacia. She was managed successfully with excision of tumor and reconstruction. The biochemical parameters improved drastically within 5 days and fracture healed in 6 weeks' time. Conclusion TIO is a debilitating disease with significant morbidity due to prolonged onset to diagnosis interval and difficulty in localizing the causative tumor. So thorou...
The Journal of Clinical Endocrinology & Metabolism, 2000
Tumor-induced osteomalacia is characterized by paraneoplastic defects in vitamin D metabolism, proximal renal tubular functions, and phosphate transport. The resulting hypophosphatemia can cause generalized pain and muscle weakness, which significantly affect the quality of life of the patients. Palliative treatment with calcium, vitamin D, and phosphate replacement is indicated for patients in whom the causative tumor cannot be completely resected. In this report we describe a case of tumor-induced osteomalacia in whom adequate oral doses of phosphate could not be used because of gastrointestinal side-effects. Long term (3-6 months) iv phosphate infusion delivered by ambulatory infusion pumps in combination with oral calcium and vitamin D was used successfully to decrease pain and increase muscle strength. Careful monitoring of serum calcium, phosphate, and creatinine levels and reliable microinfusion technology have allowed the long term use of iv phosphate infusion without serious morbidity. This patient received repeated (three times) phosphate infusions over 8 yr, resulting in laboratory and symptomatic improvement after each course. However, this patient did suffer two episodes of central venous catheter-related infection. Because of potentially serious complications, such as severe hypocalcemia, calcified right ventricular thrombi, and nephrocalcinosis, long term iv phosphate infusion should be reserved for patients who cannot tolerate adequate doses of oral phosphate and for whom the benefits outweigh the risks. (J Clin Endocrinol Metab 85: 549 -555, 2000)
Tumor-induced osteomalacia: experience from three tertiary care centers in India
Endocrine Connections
Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by recalcitrant hypophosphatemia. Reports from the Indian subcontinent are scarce, with most being single center experiences involving few patients. Herein, we conducted a retrospective analysis of 30 patients of TIO diagnosed at three tertiary care hospitals in India. Patients with persistent hypophosphatemia (despite correction of hypovitaminosis D), normocalcemia, elevated alkaline phosphatase, low TmP/GFR and elevated or ‘inappropriately normal’ FGF23 levels were labeled as having TIO. They were sequentially subjected to functional followed by anatomical imaging. Patients with a well-localized tumor underwent excision; others were put on phosphorous and calcitriol supplementation. The mean age at presentation was 39.6 years with female:male ratio of 3:2. Bone pain (83.3%) and proximal myopathy (70%) were the chief complaints; 40% of cases had fractures. The mean delay in diagnosis was 3.8 years. Tum...
Tumor-Induced Osteomalacia: Clinical and Basic Studies
Journal of Bone and Mineral Research, 1997
A patient with classic clinical and biochemical features of tumor-induced osteomalacia (hypophosphatemia, phosphaturia, and undetectable serum concentrations of 1,25-dihydroxyvitamin D [1,25(OH) 2 D]) was studied before and after resection of a benign extraskeletal chondroma from the plantar surface of the foot. Presurgical laboratory evaluation was notable for normal serum concentrations of calcium, intact parathyroid hormone (PTH), parathyroid hormone-related protein (PTHrP), and osteocalcin, increased serum alkaline phosphatase activity, and frankly elevated urinary cyclic adenosine monophosphate (cAMP) and pyridinium cross-link excretion. Quantitative histomorphometry showed severe osteomalacia and deep erosions of the cancellous surface by active osteoclasts. After resection, serum 1,25(OH) 2 D normalized within 24 h, while renal tubular phosphorus reabsorption and serum phosphorus did not normalize until days 2 and 3, respectively; serum Ca declined slightly, and serum intact PTH, osteocalcin, and urinary pyridinium cross-link excretion increased dramatically. Urinary cAMP excretion declined immediately after resection and then began to increase concomitant with the increase in serum intact PTH. A second bone biopsy taken 3 months after resection demonstrated complete resolution of the osteomalacia, increased mineral apposition rate (1.09 /day), resorption surface (9.2%), mineralizing surface (71%), and bone formation rate (0.83 mm 3 /mm 2 /day), and marked decreases in cancellous bone volume (13.1%) and trabecular connectivity compared with the first biopsy. Tumor extracts did not affect phosphate transport in renal epithelial cell lines or 1␣-hydroxylase activity in a myelomonocytic cell line. The patient's course suggests that the abnormal 1,25(OH) 2 D and phosphorus metabolism is due to a tumor product that may be acting via stimulation of adenylate cyclase activity. Increased bone resorption prior to surgical resection suggests that the tumor may also produce an osteoclast activator. The rise in resorption surface and pyridinium cross-link excretion, increase in serum osteocalcin and bone mineralization, normalization of osteoid width, and fall in cancellous bone volume after resection are consistent with healing of osteomalacia by rapid remodeling. (J Bone Miner Res 1997; 12:1502-1511)
Endokrynologia Polska
The clinical manifestation of oncogenic osteomalacia includes bone pain, pathological fractures, general fatigue and muscle weakness. Such unspecific symptoms hinder the establishment of a proper diagnosis which very often requires long-lasting investigations with many diagnostic imaging methods. Here, we discuss difficulties in the diagnosis of oncogenic osteomalacia using the example of our own clinical case: a 56 year-old woman with a history of pain in the left hip and two years of walking difficulties. A plain radiograph and CT scan revealed pathological fractures. Multiple myeloma, primary hyperparathyroidism and bone metastatic disease were excluded. Routine laboratory tests showed elevated alkaline phosphatase and a mild degree of hypophosphatemia. CT and MR imaging confirmed the presence of a pathological mass in the thorax. Tumour excision and histopathological test results revealed the diagnosis of a phosphaturic mesenchymal tumour. Our case, showing the clinical course o...
Tumor-induced osteomalacia: a case report
Arquivos Brasileiros de Endocrinologia & Metabologia, 2009
Tumor-induced osteomalacia (TIO) is a rare paraneoplasic syndrome with overproduction of fibroblast growth factor 23 as a phosphaturic agent, leading to chronic hyperphosphaturia and hypophosphatemia, associated with inappropriately normal or low levels of 1,25-dihydroxyvitamin D. Diagnosis of this disease is often challenging. The following case report described a middle-aged man with symptoms of bone pain and severe muscle weakness, who was found to have TIO. The tumor responsible for the symptoms was localized on his thigh and its resection resulted in normalization of blood chemistry and complaints. Subsequent microscopic examination revealed a phosphaturic mesenchymal tumor, mixed connective tissue type. The authors reinforce the importance of recognition of this disease, as severe disability and even death can be avoided with the surgical removal of the causative tumor.