The Search for Primary Tumor in a Patient With Oncogenic Osteomalacia

Agrawal, Archi; Jambhekar, Nirmala A.; Sarathi, Vijaya; Jagtap, Varsha S.; Agarwal, Manish; Kulkarni, Mukta; Asopa, Ramesh; Shah, Nalini

doi:10.1097/rlu.0b013e3181f49e69

Cited by 14 publications

(8 citation statements)

References 11 publications

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“…Because lymphocytes can express octreotide receptors, nonspecific uptake attributable to inflammatory tissues, fractures, or other tumors may cause false-positive results. Negative or false-positive 99m Tc-OCT scans necessitate further investigation using PET/CT [ 16 , 17 ]. Jadhav et al reported that 68 Ga-DOTA-TATE PET/CT may be more sensitive than 18 F–FDG PET/CT because of its high affinity for somatostatin receptors [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

Treatment and outcomes of tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors: retrospective review of 12 patients

Zuo

Wang

et al. 2017

BMC Musculoskelet Disord

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BackgroundTumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by severe hypophosphatemia and osteomalacia. Nonspecific symptoms make the diagnosis elusive. In addition, locating the responsible tumor(s) is challenging. The aim of this study was to investigate the clinical management and outcomes of TIO.MethodsThe clinical features, diagnostic procedures, treatment, and outcomes of 12 patients were reviewed retrospectively.ResultsThe cohort comprised six men and six women (mean age 45.5 ± 9.9 years, range 23–61 years). The mean duration of disease was 3.7 ± 2.6 years. All patients manifested progressive bone pain, muscle weakness, and/or difficulty walking. Serum phosphorus concentrations were low in all patients (mean 0.42 ± 0.12 mmol/L). Technetium-99m octreotide scintigraphy was performed in 11 patients and showed lesions in the right distal femur, left femoral head, and right tibial plateau, respectively, in three patients. Magnetic resonance imaging (MRI) was negative for lesions in one patient. Two patients underwent biopsies that showed negative histopathology. Two patients, at 2 years and 8 months, respectively, after having negative technetium-99m octreotide studies, underwent 18F–fluorodeoxyglucose positron emission tomography/computed tomography (CT), which revealed lesions in the sacrum and soft tissue of the left palm, respectively. One tumor was detected by CT and MRI. Overall, lesion sites were the head (two patients, 16.7%), thoracic and lumbar region (two, 16.7%), pelvis (three, 25%), lower limbs (four, 33.3%), and upper limbs (one, 8.3%). All patients underwent surgery, and histopathology showed phosphaturic mesenchymal tumors in each. Postoperatively, serum phosphorus concentrations normalized within 2–7 days in 11 patients. With follow-ups of 1–41 months, surgery was effective in 10 patients. One patient developed local recurrence and another had metastases.ConclusionsLocating tumors responsible for tumor-induced osteomalacia is often challenging. Although complete tumor resection confers a good prognosis in most patients, surveillance for recurrence and metastasis is necessary. Before surgery or when surgery is not indicated, oral phosphate can alleviate symptoms and metabolic imbalance.Electronic supplementary materialThe online version of this article (10.1186/s12891-017-1756-1) contains supplementary material, which is available to authorized users.

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Section: Discussionmentioning

confidence: 99%

Treatment and outcomes of tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors: retrospective review of 12 patients

Zuo

Wang

et al. 2017

BMC Musculoskelet Disord

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“…High-resolution magnetic resonance imaging (MRI) of the whole body is the currently proposed method of choice to confirm the location of the tumor. F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) is a very sensitive method [9, 10, 13] but also nonspecific. Mesenchymal tumors often express somatostatin receptors [14]; therefore, octreotide scintigraphy is another functional imaging modality.…”

Section: Introductionmentioning

confidence: 99%

Tumor-Induced Osteomalacia: Increased Level of FGF-23 in a Patient with a Phosphaturic Mesenchymal Tumor at the Tibia Expressing Periostin

Hautmann

Schroeder

Wild

et al. 2014

Case Reports in Endocrinology

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In our case, a 45-year-old male patient had multiple fractures accompanied by hypophosphatemia. FGF-23 levels were significantly increased, and total body magnetic resonance imaging (MRI) revealed a tumor mass located at the distal tibia leading to the diagnosis of tumor-induced osteomalacia (TIO). After resection of the tumor, hypophosphatemia and the increased levels of FGF-23 normalized within a few days. Subsequent microscopic examination and immunohistochemical analysis revealed a phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) showing a positive expression of somatostatin receptor 2A (SSTR2A), CD68, and Periostin. Electron microscopy demonstrated a poorly differentiated mesenchymal tumor with a multifocal giant cell component and evidence of neurosecretory-granules. However, the resected margins showed no tumor-free tissue, and therefore a subsequent postoperative radiotherapy was performed. The patient is still in complete remission after 34 months. Tumor resection of PMTMCTs is the therapy of choice. Subsequent radiotherapy in case of incompletely resected tumors can be an important option to avoid recurrence or metastasis even though this occurs rarely. The prognostic value of expression of Periostin has to be evaluated more precisely in a larger series of patients with TIO.

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“…[ 4 , 5 ] As PMTMCT tumors are typically rich in blood flow, F-18FDG PET/CT is a very sensitive, yet nonspecific, diagnostic tool. [ 6 ] High-resolution whole-body MRI imaging can effectively confirm the location of tumor. [ 5 , 7 ] In addition, studies have shown that 68Ga-DOTATATE PET is a promising and diagnostic tool for locating TIO tumors.…”

Section: Discussionmentioning

confidence: 99%

A case report of phosphaturic mesenchymal tumor-induced osteomalacia

Wang²,

Ruan³

et al. 2017

Medicine

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Rationale:Tumor-induced osteomalacia (TIO) is a rare and often misdiagnosed syndrome. Surgical resection is currently the first line treatment for TIO.Patient concerns:Here we report the case of a 49-year-old woman presented with intermittent pain in the right chest and bilateral hip that had persisted for over two years.Diagnoses:She was diagnosed of TIO caused by a phosphaturic mesenchymal tumor based on the following examinations. Laboratory tests revealed high serum alkaline phosphatase, high urinary phosphorus, hypophosphatemia and normal serum calcium levels. 18-FDG PET/CT indicated a systemic multi-site symmetrical pseudo fracture and a tumor in the 7th right rib.Interventions:Curettage of the tumor was performed, and pathological analysis also confirmed our diagnoses as a phosphaturic mesenchymal tumor.Outcomes:At seven months post-surgery, the symptoms were relieved, proximal muscle strength was improved and serum levels of phosphorus and alkaline phosphatase normalized. The bilateral femoral neck and bilateral pubic bone fractures were blurred in the pelvic plain X-ray, suggesting that the fracture was healing.Lessons:This case report strengthened the importance of recognition of this rare disease to avoid delay of diagnosis and surgical removal of the causative tumor is recommended.

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The Search for Primary Tumor in a Patient With Oncogenic Osteomalacia

Cited by 14 publications

References 11 publications

Treatment and outcomes of tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors: retrospective review of 12 patients

Treatment and outcomes of tumor-induced osteomalacia associated with phosphaturic mesenchymal tumors: retrospective review of 12 patients

Tumor-Induced Osteomalacia: Increased Level of FGF-23 in a Patient with a Phosphaturic Mesenchymal Tumor at the Tibia Expressing Periostin

A case report of phosphaturic mesenchymal tumor-induced osteomalacia

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