Category: Calcium/Bone Disorders

Type: e-Poster

Monitor: 9

9 - ONCOGENOUS OSTEOMALACIA DUE TO BENIGN MESENCHYMAL TUMOR OF RIGHT HIP: RESIDUAL TUMOR - REGROWTH - REPEAT SURGERY - CURE [20 YEARS FOLLOW UP]

Thursday, Apr 25
11:30 AM – 12:00 PM

Objective :

To describe a case of adult onset hypophosphatemic osteomalacia: Oncogenous osteomalacia due to mesenchymal tumor right hip residual tumor- regrowth – recurrence – repeat surgery and cure!!


Methods :

Age 42: female; Diagnosis Adult onset hypophosphatemic osteomalacia and metabolic myopathy [Referred from neurology to endocrinology: progressive severe muscle/ bone pains, inability to walk, even unable to bear weight]. S Calcium: 8.8 mg/dl [8.2-10.5]; S Phosphorus: 1.7 mg/dl [2.7-4.5]; S Alk phos: 309 u/l [40-139]; S PTH: 59 pg/ml [12-72]. Bone scan – Tc: Metabolic bone disease. Oncogenous osteomalacia considered – but PET CT unavailable in Bangalore / India.


Dramatic improvement with phosphate and active vitamin D; correction of hypophosphatemia; regained full ability to walk independently, muscle/bone pains disappeared.


Results :

Age 54: FDG PET – CT whole body: Soft tissue mass [33 x 23 mm] right upper medial thigh; mesenchymal tumor [FDG and Dotonoc positive].

Surgery: Excision [near total??] + Curettage of the soft tissue mass [3 x 3 cm]; Very difficult surgery due to the deep anatomical location, local tumor “infiltration”, adherence to hip joint capsule. HPE: Mesenchymal tumor:  2.5 x 2.5 x 1 cm; Cellular tumor with sheets of polygonal cells arranged about ectatic vascular spaces. Focal areas of cartilaginous metaplasia with ‘grundgy’ calcification. Nuclear features bland.


Post-Operative course:  “Persistent hypophosphatemia [1.7-2.5 on oral phosphate], Oral phosphate continued.


Age 58: S Calcium: 9.6; S Phosphorus: 2.5; S Alk phos: 155 u/l; S PTH: 411 pg/ml [12-72].


FDG PET – CT: Soft tissue mass [10 x 18 mm = smaller] in right upper medial thigh– recurrent mesenchymal tumor.


Age 61: FDG PET – CT 68Ga Dotonoc: Same tumor 19 x 9 mm.  Surgery: Excision biopsy ? Curative; mesh repair[entire fascia, tissue within the obturator canal; part of iliac, superior ramus, and periosteum].


Follow up: Completely asymptomatic; no need for oral phosphorus; S Calcium 11.1 [10.0 on Cinacalcet]; S Phosphorus=3.1; S PTH= 110; S creatinine 0.52 mg/dl.


Discussion :

These tumors typically follow a benign clinical course and even in the rare malignant cases local recurrence occurs in in <5%. A radical surgical approach is often necessary. 


Conclusion :

Somatostatin analogues can provide partial suppression of FGF 23 in residual/ recurrent tumors.  In difficult situations regional venous sampling for FGF23 levels to try and focus the area of concentration of CT scanning, has been useful.  In addition to the standard therapeutic maneuvers, another potential therapy is radiofrequency ablation, if the tumor is in a difficult location.

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Jayalakshmi T

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty

Specialties: Endocrinology, Diabetes, Internal Medicine,
Clinical Interests: Diabetes mellitus, Cardiovascular Health, Thyroid, In Patient Diabetes Care, Endocrinology Metabolic emergencies and critical care

Kavitha Muniraj

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty; Director Medical ; Director Research; Trustee
Specialties: Endocrinology Diabetes; Clinical Research
Clinical Interests: Pituitary Adrenal disorders; Bone health; Cardiovascular Health; Thyroid dysfunction; Diabetes Pharmacotherapy; Clinical Pharmacology; Pharmacovigilance; Clinical research Safety and Audits

Reshma B. Vijay

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty
Specialties: Endocrinology Diabetes and Metabolism
Clinical Interests: Pediatric Endocrinology; Diabetes mellitus; Insulin Pumps, Continuous Glucose Monitoring and Artificial Pancreas; Growth and Puberty; Thyroid; Pituitary Adrenal disorders

Vasanthi Nath

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty
Specialties: Endocrinology, Diabetes, Internal Medicine, Geriatrics
Clinical Interests: Neuroendocrinology and Neurometabolism, Metabolic Myopathies; Thyroid tumors, Geriatric Endocrinology, Inpatient Diabetes Care and Endocrine Emergencies; Type 1 diabetes across ages

Chandraprabha S

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty
Specialties: Endocrinology Diabetes Metabolism
Clinical Interests: Pregnancy Diabetes, Pregnancy Thyroid Dysfunction, Women Health, Geriatric Endocrinology, Diabetes mellitus and Mental Health, Spiritual Medicine

Babitha D. T

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty
Specialties: Endocrinology, Diabetes
Clinical Interests: Endocrine disorders of pregnancy, Pregnancy Diabetes, Cardiovascular Health, Thyroid, Inpatient diabetes care and endocrine metabolic emergencies

Muralidhara Krishna

Consultant Faculty Endocrinology Diabetes Biochemistry
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, Karnataka, India

Designation: Consultant and Faculty: Endocrinology Diabetes Metabolism; Associate Professor: Clinical Biochemistry and Laboratory Medicine
Specialties: Endocrinology Diabetes Metabolism; Clinical Biochemistry
Clinical Interests: Diabetes mellitus; Thyroid Disorders; Obesity; Dyslipidemia.

Nidhi Garg

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
bangalore, Karnataka, India

Designation: Senior Consultant and Faculty
Specialties: Diabetes and Metabolism
Clinical Interests: Comprehensive diabetes care, In-patient diabetes and metabolic care, Perioperative diabetes management, Diabetes and endocrine emergencies – Metabolic Intensive Care, Obesity management, Lipids and preventive cardiology, Gestational diabetes, Thyroid disorders, Type 1 diabetes across ages

Apoorva Govind

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center
Bangalore, India

Designation: (a) Fellow: Endocrinology Diabetes Medicine, Samatvam Endocrinology Diabetes Centre and Jnana Sanjeevini Medical Centre and Diabetes Hospital ; (b) Co Investigator – Clinical Research, Samatvam Science and Research Center

Specialties: Endocrinology, Diabetes, Internal Medicine

Siddartha Dinesha

Resident, Medicine Diabetes Endocrinology
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center

Designation: Resident: Medicine; Endocrinology Diabetes, Samatvam Endocrinology Diabetes Centre and Jnana Sanjeevini Medical Centre and Diabetes Hospital
Specialties: Endocrinology, Diabetes, Internal Medicine

MD Chitra

Consultant Faculty Endocrinology Diabetes
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center

Designation: Consultant and Faculty; Trustee
Specialties: Endocrinology Diabetes; Clinical Research
Clinical Interests: Diabetes, Obesity, Dyslipidemia, Osteoporosis, Pituitary Adrenal disorders; Bone health; Cardiovascular Health; Thyroid dysfunction;

Vasudha Srikanth


India

Sathvik Reddy Chinna Bainagari

Resident Doctor
Samatvam jnana sanjeevini Diabetes hospital and medical center
Chittoor, Andhra Pradesh, India

Madhumati S Vaishnav

Scientist Faculty
Samatvam Jnana Sanjeevini Diabetes Hospital and Medical Center; CENSE Indian Institute of Science, India

B.Sc (Microbiology), University of Pune, India.
M.Sc (Microbiology), University of Pune, India.
Post Graduate Diploma in Clinical Research and Clinical Data Management, University of Pune, India
CDE (Certified Diabetes Educator - Counselor), Samatvam Institute of Medical Sciences, India

Keerthana Haridas

Namya Gaekwad

Sri Srikanta