Needle(s) in the Haystack—Synchronous Multifocal Tumor-Induced Osteomalacia

Annamalai, Anand K; Sampathkumar, Krishnaswamy; Kane, Shubhada; Shetty, Nitin; Kulkarni, Suyash; Rangarajan, Venkatesh; Purandare, Nilendu; Pai, Prathamesh; Mahuvakar, Ankit D.; Radhakrishnan, S.; Suriyakumar, G; Puri, V.; Aram, Subramaniam; Gopalakrishnan, Chandrasekhar; Chelian, Mathirajan; Srinivasan, Keerthana; Gill, Anthony J.; Gurnell, Mark; Clifton‐Bligh, Roderick

doi:10.1210/jc.2015-3854

Cited by 11 publications

(4 citation statements)

References 9 publications

(7 reference statements)

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“…Although recent literature supports no clear anatomical predilection for the tumors (11), we found a striking lower limb predominance (72%); thighs being the most common site. Two of our patients had multifocal benign tumors (patients 19 and 21), something that has rarely been reported (16, 21, 40, 41). One of them (patient 21), a 42-year-old male (16), had an FDG-avid nodule in the right leg (measuring 0.9 × 0.6 × 1.0 cm) and a synchronous non-FDG-avid nodule in the left thigh that was picked up on MRI (measuring 0.8 × 0.7 cm).…”

Section: Discussionmentioning

confidence: 62%

“…Fortunately, complete tumoral excision leads to rapid normalization of biochemical parameters and resolution of symptoms (14). Around 300 cases of TIO have been reported worldwide, however reports from the Indian subcontinent are limited (2, 15, 16, 17, 18, 19, 20, 21). Herein, we describe the clinical features, biochemical parameters, imaging modalities and the treatment outcome in 30 patients of TIO diagnosed at three tertiary care hospitals in India.…”

Section: Introductionmentioning

confidence: 99%

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Tumor-induced osteomalacia: experience from three tertiary care centers in India

Pal

Bhadada

Singhare

et al. 2019

Endocrine Connections

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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. Tumors were clinically detectable in four patients (13.3%). The mean serum phosphate was 0.50 mmol/L with a median serum FGF23 level of 518 RU/mL. Somatostatin receptor-based scintigraphy was found to be superior to FDG-PET in tumor localization. Lower extremities were the most common site of the tumor (72%). Tumor size was positively correlated with serum FGF23 levels. Twenty-two patients underwent tumor resection and 16 of them had phosphaturic mesenchymal tumors. Surgical excision led to cure in 72.7% of patients whereas disease persistence and disease recurrence were seen in 18.2% and 9.1% of cases, respectively. At the last follow-up, serum phosphate in the surgically treated group was significantly higher than in the medically managed group.

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Section: Discussionmentioning

confidence: 62%

Section: Introductionmentioning

confidence: 99%

Tumor-induced osteomalacia: experience from three tertiary care centers in India

Pal

Bhadada

Singhare

et al. 2019

Endocrine Connections

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“…Here we present a detailed review of published English literature for TIO cases involving head and neck region ( n = 163) (5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, …”

Section: Discussionmentioning

confidence: 99%

Tumor induced osteomalacia in head and neck region: single center experience and systematic review

Shah

Lila

Jadhav

et al. 2019

Endocrine Connections

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Tumor-induced osteomalacia in the head and neck region remains a challenging diagnosis to manage. Literature pertaining to management and outcome details remains sparse. We describe two cohorts: cohort 1 included seven patients from a single center in Western India with tumors located in paranasal sinuses (n = 3), intracranial (n = 2) and maxilla (n = 2). The unique features from our series is the management of persistent disease with radiation therapy (n = 2) and peptide receptor radionuclide therapy (PRRT) (n = 1). Cohort two has 163 patients identified from 109 publications for systematic review. Paranasal sinuses, mandible, intracranial disease, maxilla and oral cavity, in descending order, are reportedly common tumor sites. Within this cohort, mean age was 46 ± 14 years at presentation with 44.1% having local symptoms. Duration of symptoms varied from 1 to 240 months. Pre-surgery mean serum phosphorus was 1.4 ± 0.4 mg/dL and median FGF-23 levels were 3.6 (IQR:1.8–6.8) times of normal upper limit of normal. Majority (97.5%) were managed primarily with surgical excision; however, primary radiotherapy (n = 2) and surgery combined with radiotherapy (n = 2) were also reported. Twenty patients had persistent disease while nine patients had recurrence, more commonly noted with intracranial and oral cavity tumors. Surgery was the most common second mode of treatment employed succeeded by radiotherapy. Four patients had metastatic disease. The most common histopathological diagnosis reported is PMT mixed connective tissue, while the newer terminology ‘PMT mixed epithelial and connective tissue type’ has been described in 15 patients.

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“…The most common sites include extremities and acral sites but soft tissue organs like the liver or heart have not been reported to be involved yet [ 3 , 5 , 6 , 14 , 29 ]. Rarely PMTs present as multifocal disease [ [30] , [31] , [32] , [33] ].…”

Section: Clinical Description and Evaluationmentioning

confidence: 99%

Tumor-induced osteomalacia

Yin

et al. 2018

Osteoporosis and Sarcopenia

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Tumor-induced osteomalacia (TIO), also known as oncogenic osteomalacia, is a rare paraneoplastic syndrome characterized by hypophosphatemia resulting from decreased tubular phosphate reabsorption, with a low or inappropriately normal level of active vitamin D. The culprit tumors of TIO could produce fibroblast growth factor 23 which plays a role in regulating renal Pi handling and 25-hydroxyvitamin D 1α-hydroxylase activity. Chronic hypophosphatemia could eventually lead to inadequate bone mineralization, presenting as osteomalacia. The diagnosis should be considered when patients manifest as hypophosphatemia and osteomalacia, or rickets and needs to be differentiated from other disorders of phosphate metabolism, such as the inhereditary diseases like X-linked hypophosphataemic rickets, autosomal dominant hypophosphataemic rickets, autosomal recessive hypophosphataemic rickets and acquired diseases like vitamin D deficiency. Localization of responsible tumors could be rather difficult since the vast majority are very small and could be everywhere in the body. A combination of thorough physical examination, laboratory tests and imaging techniques should be applied and sometimes a venous sampling may come into handy. The technology of somatostatin-receptor functional scintigraphy markedly facilitates the localization of TIO tumor. Patients undergoing complete removal of the causative neoplasm generally have favorable prognoses while a few have been reported to suffer from recurrence and metastasis. For those undetectable or unresectable cases, phosphate supplements and active vitamin D should be administrated and curative intended radiotherapy or ablation is optional.

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Needle(s) in the Haystack—Synchronous Multifocal Tumor-Induced Osteomalacia

Cited by 11 publications

References 9 publications

Tumor-induced osteomalacia: experience from three tertiary care centers in India

Tumor-induced osteomalacia: experience from three tertiary care centers in India

Tumor induced osteomalacia in head and neck region: single center experience and systematic review

Tumor-induced osteomalacia

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