Oncogenic osteomalacia due to phosphaturic mesenchymal tumour in the upper thoracic spine

2020 ◽  
Vol 13 (12) ◽  
pp. e238209
Author(s):  
Bhavuk Garg ◽  
Nishank Mehta ◽  
Archit Goyal ◽  
Rajesh Khadgawat
Keyword(s):  
Surgical Resection ◽  
Functional Imaging ◽  
Fibroblast Growth Factor 23 ◽  
Serum Vitamin ◽  
Lower Limbs ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumour ◽  
Serum Vitamin D ◽  
Complete Surgical Resection ◽  
Phosphaturic Mesenchymal Tumour

Oncogenic osteomalacia (OO) is an uncommon paraneoplastic syndrome occurring due to the presence of a tumour that oversecretes fibroblast growth factor-23, which impairs renal phosphate handling. In most cases, the tumour is a morphologically distinct entity called ‘phosphaturic mesenchymal tumour’ (PMT). Spinal tumours causing OO are exceedingly rare. A 55-year-old man presented with multiple bone pain and proximal muscle weakness in the lower limbs. The constellation of biochemical findings (hypophosphataemia, normocalcaemia, increased alkaline phosphatase, low–normal serum vitamin D and hyperphosphaturia) with radiographical rarefaction of the skeleton and pseudofractures led us to consider OO as a possibility. Functional imaging (68Ga DOTA-NOC positron emission tomography/CT scan) localised the tumour to the D2 vertebra. Complete surgical resection led to resolution of symptoms, improved ambulatory status, normalisation of biochemical parameters and healing of pseudofractures. PMT should be considered in the differential diagnosis of hypophosphataemic osteomalacia with hyperphosphaturia. Tumour localisation with functional imaging and complete surgical resection produces satisfactory outcome.

Phosphaturic Mesenchymal Tumors of the Sinonasal Area and Skull Base: Experience at a Single Institution

2021 ◽  
pp. 000348942110374
Author(s):  
Davis P. Argersinger ◽  
Catherine T. Haring ◽  
John E. Hanks ◽  
Kevin J. Kovatch ◽  
S. Ahmed Ali ◽  
...  
Keyword(s):  
Skull Base ◽  
Surgical Resection ◽  
Medical Center ◽  
Fibroblast Growth Factor 23 ◽  
Academic Medical Center ◽  
Relevant Literature ◽  
Case Series ◽  
Radiological Evidence ◽  
Lateral Skull Base ◽  
Complete Surgical Resection

Objectives: Phosphaturic mesenchymal tumor (PMT) is a rare, polymorphous neoplasm with a highly variable presentation and natural history and unpredictable clinical course. The primary objective was to describe our clinical experience with and management of 4 markedly different cases of sinonasal and skull base PMT. Methods: A retrospective case series with chart review, and relevant literature review, was performed at a tertiary academic medical center between 1998 and 2020. Adult patients treated for PMTs of the sinonasal area and skull base were included. Our main outcome measures included postoperative laboratory findings and radiological evidence of disease remission. Results: Four patients (2 Males, 2 Females; Mean Age: 63.5 years) with PMTs of the skull base have been managed at our institution since 1998. Patient presentations varied, ranging from severe phosphorus wasting and osteoporosis to symptoms secondary to mass effect, including nasal obstruction and rhinorrhea. All 4 patients were eventually found to have elevated levels of fibroblast growth factor 23. Tumors were located in the sinonasal area (right frontal sinus, right ethmoid sinus, and right nasal cavity, respectively) in 3 patients and in the lateral skull base (right jugular foramen) in 1 patient. All 4 patients underwent complete surgical resection of their tumors. PMT tissue pathology was confirmed in all cases. Gross total resection was achieved in all patients. There was no chemical or radiological evidence of disease recurrence in any patients at follow-up. Conclusions: The presentation of skull base PMT is variable, and it may mimic other mass pathologies of the head and neck. Complete surgical resection with negative margins is potentially curative.

scholarly journals Infantile Blount’s Disease: A case report of 3 years old female of Zhob, Pakistan

2021 ◽  
Vol 15 (8) ◽  
pp. 1993-1995
Author(s):  
Sumera Akram ◽  
Muhammad Ahmed Khan
Keyword(s):  
Vitamin D ◽  
Serum Alkaline Phosphatase ◽  
Early Stage ◽  
Clinical Signs ◽  
Serum Vitamin ◽  
Disease Process ◽  
Lower Limbs ◽  
Genu Varum ◽  
Blount’S Disease ◽  
Serum Vitamin D

Background; Blount’s disease is a rare developmental disorder of children which causes progressive bowing of lower limbs. The term “Blount” was named after American orthopedic surgeon “Walter Putnam Blount” who first described this condition. The etiology of Blount’s disease is unknown but believed to be multifactorial. Various predisposing factors have been attributed including obesity, early walking, race, pre-existing varus, increased pressure on growth plate and nutrition. Blount’s disease has been suggested to be more frequent in African, Afro-american populations. Blount’s disease has to be differentiated from physiological bowing (physiologic genu varum) and rickets. Early diagnosis and treatment of Blount’s disease is essential as the disease process is reversible in early stage. Case; A three years old female child was brought by her mother with complaint of progressive bowing of both lower limbs for last one year. She achieved her milestones at appropriate age and started walking at 11 months of age without support. On examination, her height was 90 cms (at 10th centile) and weight was 17 kgs (at 90th centile). BMI (body mass index) was 20.9 (obese). There were no clinical signs or symptoms of rickets i.e frontal bossing, wide wrist, rachitic rosary, carpopedal spasm, fits or muscle weakness etc. Roentgenogram showed tibia in varus with a peculiar beak at metaphysic and raised metaphyseal-diaphyseal angle (>16 degrees). Serum calcium and serum vitamin D (25-hydroxy vitamin D) were normal. Serum alkaline phosphatase level was raised. Keeping in view typical history, examination findings and radiological epiphyseal beaking along with raised metaphyseal-diaphyseal angle, diagnosis of Infantile Blount’s disease was made. Conclusion; The clinicians should have a high suspicion of infantile blount’s disease when a child, more than 3 year’s age presents with severe varus deformity at proximal tibia with typical radiological findings. Characteristic radiologic findings along with history and examination help to distinguish it from physiologic bowing (physiologic genu varum) and rickets. Keywords; Blount’s disease, Physiologic genu varum, Rickets, Tibia vara, Osteochondrosis deformans tibiae

Oncogenic osteomalacia due to phosphaturic mesenchymal tumour in infratemporal fossa

2010 ◽  
Vol 61 (5) ◽  
pp. 392-394
Author(s):  
Guillem Viscasillas ◽  
Javier Maiz ◽  
Xavier Lao ◽  
Christiane Zschaeck ◽  
Juan José Sanz
Keyword(s):  
Infratemporal Fossa ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumour ◽  
Phosphaturic Mesenchymal Tumour

scholarly journals Oncogenic osteomalacia secondary to hemangiopericytoma: a case report

2021 ◽  
Vol 8 (1) ◽  
pp. 115
Author(s):  
Aamir Matin ◽  
Sushant Sopan Tuse
Keyword(s):  
Serum Levels ◽  
Proximal Muscle ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumour ◽  
Complete Excision ◽  
Post Surgery ◽  
Pet Ct ◽  
Fgf 23 ◽  
Very High ◽  
Phosphaturic Mesenchymal Tumour

<p>We are going to present a case of hypo-phosphatemic rickets secondary to phosphaturic mesenchymal tumour who came with complaints of proximal muscle weakness which limited his effort tolerance and activities of daily life like standing from squatting position and rib pain. His FGF-23 levels were very high above normal levels and PET CT revealed a well-defined enhancing lesion abutting femoral neurovascular bundle. After consultation with endocrinologist, we have d done complete excision of the mass. Post-surgery all symptoms were relieved, proximal muscle strength improved gradually and serum levels of phosphorus, ALP and FGF-23 came back to normal.</p>

Oncogenic osteomalacia: case report and review of head and neck associated tumours

1998 ◽  
Vol 112 (4) ◽  
pp. 389-392 ◽  
Author(s):  
X. Gonzalez-Compta ◽  
M. Mañós-Pujol ◽  
M. Foglia-Fernandez ◽  
E. Peral ◽  
E. Condom ◽  
...  
Keyword(s):  
Case Report ◽  
Head And Neck ◽  
Surgical Approach ◽  
Mineral Metabolism ◽  
Oncogenic Osteomalacia ◽  
Mesenchymal Tumour ◽  
Aggressive Surgical Approach ◽  
Vascular Component ◽  
Phosphaturic Mesenchymal Tumour

AbstractOncogenic osteomalacia is an uncommon syndrome characterized by mineral metabolism abnormalities that disappear after the resection of an associated tumour. Head and neck is the second most frequent location of these tumours. We describe a case with an ethmoido-frontal phosphaturic mesenchymal tumour and review oncogenic osteomalacia-associated tumours. Among 21 cases found, 57 per cent affected the sinonasal area and 20 per cent the mandible. The diagnosis of the tumour lasted a mean of 4.7 years from the onset of osteomalacia, and most of them showed a significant vascular component. An aggressive surgical approach is recommended.

Clinical factors correlated with vitamin D deficiency in patients with obesity scheduled for bariatric surgery: A single center experience

2020 ◽  
Vol 90 (3-4) ◽  
pp. 346-352
Author(s):  
Vincenzo Pilone ◽  
Salvatore Tramontano ◽  
Carmen Cutolo ◽  
Federica Marchese ◽  
Antonio Maria Pagano ◽  
...  
Keyword(s):  
Bariatric Surgery ◽  
Vitamin D ◽  
Vitamin D Deficiency ◽  
Serum Vitamin ◽  
Inverse Correlation ◽  
Model Assessment ◽  
Cross Sectional ◽  
Serum Vitamin D ◽  
Female Sex ◽  
Vitamin D Levels

Abstract. We aim to assess the prevalence of vitamin D deficiency (VDD) in patients scheduled for bariatric surgery (BS), and to identify factors that might be associated with VDD. We conducted a cross-sectional observational study involving all consecutive patients scheduled for BS from 2017 to 2019. The exclusion criteria were missing data for vitamin D levels, intake of vitamin D supplements in the 3 months prior to serum vitamin D determination, and renal insufficiency. A total of 206 patients (mean age and body mass index [BMI] of 34.9 ± 10.7 years, and 44.3 ± 6.99 kg/m2, respectively) met the inclusion criteria and were enrolled for data analysis. VDD (<19.9 ng/mL), severe VDD (<10 ng/mL), and vitamin D insufficiency (20–29.9 ng/mL) were present in 68.8 %, 12.5 %, and 31.2 % of patients, respectively. A significant inverse correlation was found between vitamin D levels and initial BMI, parathyroid hormone, and homeostatic model assessment of insulin resistance (r = −0.280, p < 0.05; r = −0.407, p = 0.038; r = −0.445, p = 0.005), respectively. VDD was significantly more prevalent in patients with higher BMI [−0.413 ± 0.12, CI95 % (−0.659; −0.167), p = 0.006], whereas no significant association between hypertension [−1.005 ± 1.65, CI95 % (−4.338; 2.326), p = 0.001], and diabetes type 2 (T2D) [−0.44 ± 2.20, CI95 % (−4.876; 3.986), p = 0.841] was found. We observed significant association between female sex and levels of vitamin D [6.69 ± 2.31, CI95 % (2.06; 11.33), p = 0.006]. The present study shows that in patients scheduled for BS, VDD deficiency is common and was associated with higher BMI, and female sex.

Sign in / Sign up

Export Citation Format

Share Document