A Challenging Case of Phosphaturic Mesenchymal Tumor With Primary Hyperparathyroidism

Abstract Background: Localization of tumor-induced osteomalacia (TIO) is often challenging. Primary hyperparathyroidism (HPT) following curative surgery for TIO is rarely reported. Clinical Case: A 49-year-old man presented with fragility rib fractures, generalized bone pain, and muscle weakness worsening over the past 3 years. Rheumatologic workup was negative. Initial tests showed elevated levels of parathyroid hormone (PTH) 114.1 pg/mL (14–72 pg/mL) and alkaline phosphatase (ALP) 283 IU/L (44–174 IU/L), reduced levels of 25(OH)D 16 ng/mL (30–100 ng/mL), 1,25(OH)2D 9 pg/mL (18–72 pg/mL), and phosphorus 1.6 mg/dL (2.5–4.9 mg/dL), calcium levels of 9.2 mg/dL (8.5–10.1 mg/dL), and eGFR 58 mL/min/1.73 m2. A sestamibi scan showed normal parathyroid uptake. The diagnosis was secondary HPT due to chronic kidney disease and vitamin D deficiency. The patient was treated with D3 and phosphate. During a 2-year follow-up, the patient reported improvement of pain and weakness with no additional fractures. Further investigations showed persistent hypophosphatemia with elevated urinary fractional phosphate excretion (44%, ref. <20%), indicating renal phosphate wasting. Fibroblast Growth Factor 23 (FGF23) was high, 291 RU/mL (0–180 RU/mL). DXA results were consistent with osteopenia. TIO was suspected. At a 3-year follow-up, investigations included three whole-body 18F-FDG PET-CT scans revealing several areas suspicious for tumor presence. However, multiple MRIs were inconclusive. Laboratory tests showed persistent hypophosphatemia (despite D3 and phosphate treatment), elevated FGF23 (1330 RU/mL) and PTH (274.4 pg/mL), and normal calcium, 25(OH)D, and 1,25(OH)2D. The patient subsequently underwent 68Ga DOTATATE PET-CT, which revealed a somatostatin receptor-positive lesion involving the left upper lobe of the lung. The mass was resected without complications. Histopathology was compatible with a phosphaturic mesenchymal tumor. At a 6-month postoperative follow-up, the patient reported dramatically improved symptoms with decreased weakness and pain, normal phosphate, calcium, ALP, and FGF23 (160 RU/mL) levels, while DXA results were significantly improved. Phosphorus supplementation was discontinued. At follow-up 3 years post-surgery, the patient had slowly rising PTH (126.3 pg/mL) and calcium (10.1–10.6 mg/dL) levels with normal phosphate, 25(OH)D, and FGF23 (174 RU/mL) levels. A diagnosis of primary HPT was made. Further evaluation was deemed unnecessary since the patient did not meet the criteria for surgical treatment. The development of primary HPT was considered mechanistically related to long-standing hypophosphatemia and hypovitaminosis D stimulating PTH production. Conclusion: This case report highlights the pitfalls contributing to delayed diagnosis of TIO and alerts clinicians to the potential development of primary HPT after curative surgery for TIO.

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TIO Associated with Hyperparathyroidism: A Rarity, a Rule, or a Novel HPT-PMT Syndrome—A Case Study with Literature Review

Case Reports in Endocrinology ◽

10.1155/2021/5172131 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Michael Salim ◽

Mohannad Samy Behairy ◽

Elena Barengolts

Keyword(s):

Literature Review ◽

Somatostatin Receptor ◽

Delayed Diagnosis ◽

Whole Body ◽

Mesenchymal Tumors ◽

Pet Ct ◽

Patient Reported ◽

Minimal Improvement ◽

Biochemical Evaluation

Objective. Association of primary hyperparathyroidism (pHPT) with phosphaturic mesenchymal tumors (PMT) is rarely reported. This report entertains the hypothesis of the causal association of HPT with tumor-induced osteomalacia (TIO) and of the existence of HPT-PMT syndrome. Case Presentation. A 49-year-old man presented with fragility rib fractures, generalized bone pain, and muscle weakness worsening over the past 3 years. Initial tests demonstrated hypophosphatemia and high PTH. The diagnosis of pHPT was entertained, but parathyroid scan was negative. During a 2-year follow-up, the patient reported minimal improvement of symptoms after intermittent treatment with calcitriol and phosphate. Biochemical evaluation showed persistent hypophosphatemia with renal phosphate wasting, elevated FGF23, and osteopenia on DXA scan. TIO was suspected. Multiple MRIs and whole-body FDG-PET scans were inconclusive. The patient subsequently underwent 68Ga-DOTATATE PET-CT, which revealed a somatostatin receptor-positive lesion in the lung. The resected mass was confirmed as PMT. The patient had dramatically improved symptoms, normal phosphate, calcium, and FGF23. During follow-up over 3 years postsurgery, the patient had slowly rising calcium and persistently elevated PTH. Conclusion. The debate whether the patient had pHPT or tertiary HPT prompted literature review showing that aberrant genes including FGFR1, FGF1, fibronectin 1, and Klotho were mechanistically involved in the HPT-PMT association. This case highlights the pitfalls contributing to delayed diagnosis and treatment of TIO and hypothesizes the association between pHPT and PMT.

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SAT-LB308 Primary Hyperparathyroidism and Meningioma as a Part of Multiple Endocrine Neoplasm Type 1 (MEN Type 1)

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.2279 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Shady Ibrahim ElEbrashy ◽

ElRefaay Ehab

Keyword(s):

Primary Hyperparathyroidism ◽

Parathyroid Adenoma ◽

Histopathological Examination ◽

Surgical Excision ◽

Tracer Uptake ◽

Whole Body ◽

Genetic Studies ◽

Pet Ct

Abstract Background: Meningioma is a rare association of Multiple endocrine neoplasia type 1 (MEN 1) and very few cases has been reported in literature. Clinical Case: a 75-year-old woman showed severe headache, disturbed consciousness and convulsions. A diagnosis of cerebral meningioma was made and surgical excision was done, histopathological examination confirmed meningioma; patient was transferred to the ICU postoperatively for monitoring. Patient’s consciousness was not regained in full and remained in delirium, follow up investigations revealed: serum calcium of 13.2 mg/dl (8.5 to 10.5 mg/dl), serum sodium 141 mmol/L (135-145 mmol/L) and potassium 4.9 mmol/L (3.5-5 mmol/L), serum parathormone of 850 pg/mL (10-65 pg/mL), primary hyperparathyroidism was suspected; further investigations revealed inferior parathyroid adenoma on ultrasound which elicited focal tracer uptake on sesta-mibi parathyroid scintigraphy. Patient did excision of the lesion and was confirmed by histopathological examination to be parathyroid adenoma. Patient recovered well postoperatively, consciousness was regained and no neurological defects ware present. Genetic studies where performed and was found positive for MEN type 1 gene. Whole body Ga-DOTATATE PET/CT was then done to exclude any associated tumors and no tracer uptake was found. Patient was discharged, family members were offered genetic analysis and were counselled on the importance of screening. Conclusion: MEN type 1 can rarely present with meningiomas with symptoms very similar and easily confused with hypercalcemia and the diagnosis can be missed.

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Total Hip Arthroplasty: Minimal Clinically Important Difference and Patient Acceptable Symptom State for the Forgotten Joint Score 12

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph18052267 ◽

2021 ◽

Vol 18 (5) ◽

pp. 2267

Author(s):

Umile Giuseppe Longo ◽

Sergio De Salvatore ◽

Ilaria Piergentili ◽

Anna Indiveri ◽

Calogero Di Naro ◽

...

Keyword(s):

Total Hip Arthroplasty ◽

Hip Arthroplasty ◽

Validity And Reliability ◽

Total Hip ◽

Forgotten Joint Score ◽

Post Surgery ◽

Clinically Important Difference ◽

Patient Reported ◽

Patient Acceptable Symptom State

The Forgotten Joint Score-12 (FJS-12) is a valid patient-reported outcome measures (PROMs) used to assess prosthesis awareness during daily activities after total hip arthroplasty (THA). The minimum clinically important difference (MCID) can be defined as the smallest change or difference that is evaluated as beneficial and could change the patient’s clinical management. The patient acceptable symptom state (PASS) is considered the minimum PROMs cut-off value that corresponds to a patient’s satisfactory state of health. Despite the validity and reliability of the FJS-12 having been already demonstrated, the MCID and the PASS of this score have not previously been defined. Patients undergoing THA from January 2019 to October 2019 were assessed pre-operatively and six months post-surgery using the FJS-12, the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and the Oxford Hip Score (OHS). Pre-operative and follow-up questionnaires were completed by 50 patients. Both distribution-based approaches and anchor approaches were used to estimate MCID. The aim of this paper was to assess the MCID and PASS values of FJS-12 after total hip replacement. The FJS-12 MCID from baseline to 6 months post-operative follow-up was 17.5. The PASS calculated ranged from 69.8 to 91.7.

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High Grade tumor vs stroke. Utility of PET and MRI corregistration

ANALES RANM ◽

10.32440/ar.2021.138.02.cc01 ◽

2021 ◽

Vol 138 (138(02)) ◽

pp. 176-179

Author(s):

M.N. Cabrera-Martín ◽

P. Romero Fernández ◽

M.K. Meneses Navas ◽

P. Pérez-Segura ◽

M. Yus Fuertes ◽

...

Keyword(s):

Clinical Presentation ◽

High Grade Glioma ◽

Whole Body ◽

High Grade ◽

Mr Images ◽

Fdg Uptake ◽

Area Of Interest ◽

Pet Ct ◽

Adjacent Brain

Sometimes, the clinical presentation of a brain tumour mimics that of stroke or viceversa. In these cases it is useful to evaluate follow-up with MR. The coregistration of PET and MR images improves the diagnostic performance of both techniques. In the area of interest, FDG uptake of any degree, with reference to the adjacent brain tissue uptake, should be considered suggestive of tumor if it corresponds to brain injury in MR, even when the FDG uptake is equal to or lower than that of the normal cerebral cortex. We present a case in which coregistration of images from both techniques contributed to the differential diagnosis of stroke and high-grade glioma, whole-body PET/CT ruled out metastatic etiology, and the results led to surgery.

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Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia

Medicina ◽

10.3390/medicina56010034 ◽

2020 ◽

Vol 56 (1) ◽

pp. 34

Author(s):

Cornelia Then ◽

Evelyn Asbach ◽

Harald Bartsch ◽

Niklas Thon ◽

Christian Betz ◽

...

Keyword(s):

Growth Factor ◽

Fibroblast Growth Factor ◽

Fibroblast Growth Factor 23 ◽

Olfactory Neuroblastoma ◽

Mesenchymal Tumor ◽

Fibroblast Growth ◽

Phosphaturic Mesenchymal Tumor ◽

Oncogenic Osteomalacia ◽

Pet Ct ◽

Fgf 23

A possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional imaging. A 50-year-old woman presented with diffuse musculoskeletal pain and several fractures. Secondary causes of osteoporosis were excluded. Laboratory analysis revealed hypophosphatemia and elevated alkaline phosphatase, parathyroid hormone, and FGF-23. Thus, oncogenic osteomalacia due to neoplastic FGF-23 secretion was suspected. FDG-PET-CT and DOTATATE-PET-CT imaging demonstrated no tumor. Cranial MRI revealed a tumorous mass in the left cellulae ethmoidales. The tumor was resected and histopathological examination showed a cell-rich tumor with round to ovoid nuclei, sparse cytoplasm, and sparse matrix, resembling an olfactory neuroblastoma. Immunohistochemical analysis first led to diagnosis of olfactory neuroblastoma, which was later revised to phosphaturic mesenchymal tumor. Following the resection, FGF-23 and phosphate levels normalized. In conclusion, we here describe a patient with an FGF-23-secreting phosphaturic mesenchymal tumor with an unusual morphology. Furthermore, we emphasize diagnostic pitfalls when dealing with FGF-23-induced hypophosphatemia.

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Whole-body MR Imaging in Detecting Phosphaturic Mesenchymal Tumor (PMT) in Tumor-induced Hypophosphatemic Osteomalacia

Magnetic Resonance in Medical Sciences ◽

10.2463/mrms.2011-0006 ◽

2013 ◽

Vol 12 (1) ◽

pp. 47-52 ◽

Cited By ~ 12

Author(s):

Katsuyuki NAKANISHI ◽

Mio SAKAI ◽

Hisashi TANAKA ◽

Hideki TSUBOI ◽

Jun HASHIMOTO ◽

...

Keyword(s):

Mr Imaging ◽

Mesenchymal Tumor ◽

Whole Body ◽

Phosphaturic Mesenchymal Tumor ◽

Hypophosphatemic Osteomalacia

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Incidence and Economic Impact of Incidental Findings on 18F-FDG PET/CT Imaging

Canadian Association of Radiologists Journal ◽

10.1016/j.carj.2017.08.001 ◽

2018 ◽

Vol 69 (1) ◽

pp. 63-70 ◽

Cited By ~ 7

Author(s):

Scott J. Adams ◽

Rajan Rakheja ◽

Rhonda Bryce ◽

Paul S. Babyn

Keyword(s):

Incidental Findings ◽

Incidental Finding ◽

Ct Imaging ◽

Whole Body ◽

Ontario Health Insurance Plan ◽

Positron Emission ◽

Pet Ct ◽

Picture Archiving And Communication ◽

Fdg Pet Ct

Purpose The study sought to determine the incidence of incidental findings on whole-body positron emission tomography with computed tomography (PET/CT) imaging and the average costs of investigations to follow-up or further characterize incidental findings. Methods Imaging reports of 215 patients who underwent whole-body PET/CT imaging were retrospectively reviewed. Our provincial picture archiving and communication system was queried and patient charts were reviewed to identify all investigations performed to follow-up incidental findings within 1 year of the initial PET/CT study. Costs of follow-up imaging studies (professional and technical components) and other diagnostic tests and procedures were determined in Canadian dollars (CAD) and U.S. dollars (USD) using the 2015 Ontario Health Insurance Plan Schedule of Benefits and Fees and 2016 U.S. Medicare Physician Fee Schedule, respectively. Results At least 1 incidental finding was reported in 161 reports (74.9%). The mean number of incidental findings ranged from 0.64 in patients <45 years of age to 2.2 in patients 75 years of age and older. Seventy-five recommendations for additional investigations were made for 64 (30%) patients undergoing PET/CT imaging, and 14 of those were carried out specifically to follow-up incidental findings. Averaged across all 215 patients, the total cost of investigations recommended to follow-up incidental findings was CAD$105.51 (USD$127.56) per PET/CT study if all recommendations were acted on, and CAD$22.77 (USD$29.14) based on investigations actually performed. Conclusions As the incidence of incidental findings increases with age and a larger proportion of elderly patients is expected as population demographics change, it will be increasingly important to consider incidental findings on PET/CT imaging with standardized approaches to follow-up.

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Efficiency of whole-body 18F-FDG PET CT in detecting the cause of rising serum AFP level in post-therapeutic follow-up for HCC patients

Japanese Journal of Radiology ◽

10.1007/s11604-020-00930-8 ◽

2020 ◽

Vol 38 (5) ◽

pp. 472-479 ◽

Cited By ~ 4

Author(s):

Susan Adil Ali ◽

Darine Helmy Amin ◽

Yasser Ibrahim Abdelkhalek

Keyword(s):

Fdg Pet ◽

Whole Body ◽

Pet Ct ◽

18F Fdg ◽

Fdg Pet Ct

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Adherence to post-surgery follow-up assessment and its association with sociodemographic and disease characteristics in patients with breast cancer in Central China

BMC Cancer ◽

10.1186/s12885-020-07600-y ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Ran Feng ◽

Jingfeng Jing ◽

Xiaojun Zhang ◽

Ming Li ◽

Jinnan Gao

Keyword(s):

Breast Cancer ◽

Breast Cancer Survivors ◽

Insurance Coverage ◽

Central China ◽

Axillary Lymph ◽

Current Guideline ◽

Curative Surgery ◽

Post Surgery ◽

Treatment Characteristics

Abstract Background Follow-up after curative surgery is increasingly recognized as an important component of breast cancer care. Although current guideline regulates the follow-ups, there are no relevant studies on the adherence to it in China. This study investigated the post-surgery follow-up and explored its association with patients, tumor and treatment characteristics. Methods A total of 711 patients underwent surgical treatment in Shanxi Bethune Hospital from March 2012 to May 2018 were included in this study. Baseline sociodemographic, tumor, and treatment characteristics were obtained from the hospital electronic medical records. The post-surgery follow-up was reviewed and assessed from the patient’s follow-up examination record. Factors associated with the first three-year follow up was evaluated using logistic regression analysis. Results The annual follow-up rate after surgery decreased gradually from 67.1% at the 1st year, 60.2% at the 3rd year to 51.9% at the 4th year, and 43.5% at the 5th year. Loss of follow-up during the first 3 years after surgery was significantly associated with older age (> 65 years), lower medical insurance coverage, axillary lymph node dissection, and less intensity of systemic treatment. Conclusion A significant downtrend of annual follow-up rate for breast cancer survivors was confirmed in this study. Loss of follow-up within the first 3 years after surgery was associated with both patient’s characteristics and treatment. These results will provide evidence to help clinicians to develop tailored patient management after curative surgery.

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Tc-99m HYNIC-TOC scintigraphy in dedifferentiated thyroid cancer

BMJ Case Reports ◽

10.1136/bcr-2018-227910 ◽

2019 ◽

Vol 12 (4) ◽

pp. e227910

Author(s):

Kanhaiyalal Agrawal ◽

P Sai Sradha Patro ◽

C Preetam

Keyword(s):

Thyroid Cancer ◽

Single Photon ◽

Somatostatin Receptor ◽

Photon Emission ◽

Peptide Receptor Radionuclide Therapy ◽

Ct Imaging ◽

Whole Body ◽

Emission Computed Tomography ◽

Serum Thyroglobulin ◽

Pet Ct

There is literature evidence showing utility of somatostatin receptor (SSTR) positron emission tomography-CT (PET-CT) imaging in differentiated thyroid cancer with Thyroglobulin Elevated and Negative Iodine Scan (TENIS). These patients are less benefited with I-131 therapy and surgery remains only curable option if disease could be localised. If surgery is not feasible, other therapeutic options are not promising. However, if these patients show strongly positive SSTR imaging, then possibility of peptide receptor radionuclide therapy may be explored. As SSTR PET-CT imaging is expensive and not widely available, Technetium-99m (Tc-99m) hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC), which is a Single photon emission computed tomography (SPECT) tracer, can be used. We are documenting a case of raised serum thyroglobulin antibody and negative I-131 whole body scan with disease recurrence localised on Tc-99m HYNIC-TOC scan.

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