Oncogenic osteomalacia from nasal cavity giant cell tumor

Battoo, Azhar Jan; Salih, Surij; Unnikrishnan, Ambika Gopalakrishnan; Jojo, Annie; Bahadur, Sudhir; Iyer, Subramania; Kuriakose, Moni Abraham

doi:10.1002/hed.21562

Cited by 16 publications

(11 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This could explain the period between 1972 and 1987 when PMT was still an unrecognized entity and the unfamiliar histological features resulted in a variety of diagnoses [4]. Therefore it is important to include PMT in a differential diagnosis of mesenchymal tumors causing OO that includes HPC [63], GCTB [64], and osteosarcoma [65]. HPC shows hyalinized ''staghorn'' blood vessels that contain round to ovoid cells and which lack the grungy calcifications and the MNGCs that are found in PMT [63].…”

Section: Discussionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Qari

Hamao-Sakamoto

Fuselier

et al. 2015

Head and Neck Pathol

View full text Add to dashboard Cite

Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that secretes fibroblast growth factor-23 (FGF-23) and causes oncogenic osteomalacia. It occurs in adults with equal gender distribution and the most common location is the lower extremities, followed by the head and neck. Besides osteomalacia, the clinical presentation includes bone pain and multiple bone fractures. Microscopic features consist of spindle cells, multinucleated giant cells, and calcifications embedded in a chondromyxoid matrix. Laboratory findings indicate normal calcium and parathyroid levels, hypophosphatemia, and increased levels of FGF-23 that usually revert to normal after surgical removal. Due to its rarity, the purpose of the study was to report 2 new oral cases of PMT and to review the literature in the head and neck. The first case occurred in the gingiva and had been present for 6 years. The second case was a recurrence of a previously diagnosed PMT in the right mandible that metastasized to the lung and soft tissue. The literature review included 53 cases in the head and neck. There was a predilection for extra-oral sites (76 %) compared to intra-oral sites (24 %) with paranasal sinuses considered the most common location (38 %) followed by the mandible (15 %). There were 9 recurrences that included 3 malignant cases indicating a potentially aggressive tumor. Due to the indeterminate biological behavior of PMT and its rarity, a comprehensive evaluation of medical, laboratory, radiographic, and histological findings are crucial for a definitive diagnosis and treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Qari

Hamao-Sakamoto

Fuselier

et al. 2015

Head and Neck Pathol

View full text Add to dashboard Cite

show abstract

“…PMTMCT can occur either in the soft tissues or bone. Relevant to this discussion, a number of PMTMCT involving the craniofacial bones and sinonasal tract have been reported and illustrated [17][18][19][20][21][22][23][24][25][26][27][28][29]. Grossly, PMTMCT appear well-circumscribed, but microscopically, all tumors infiltrate the surrounding connective tissues.…”

Section: The Diverse Histologies Of Phosphaturic Mesenchymal Tumorsmentioning

confidence: 99%

Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

Brandwein-Gensler

Siegal

2012

Head and Neck Pathol

View full text Add to dashboard Cite

show abstract

“…Our patient was diagnosed to have unexplained osteomalacia three years back and was on treatment for the same. Oncogenic osteomalacia is a paraneoplastic syndrome that is attributed to a humoral factor FGF‐23 secreted by mesenchymal tumors that inhibits the renal absorption of phosphate in the proximal renal tubule . It leads to decreased serum phosphate levels and abnormal bone mineralization.…”

Section: Discussionmentioning

confidence: 99%

A 40‐Year‐Old Woman With Intracranial Bleed and Osteomalacia

Gulwani

Garg

2014

Brain Pathology

View full text Add to dashboard Cite

A 40-year-old woman presented with sudden onset of headache and altered sensorium for 2 days. In the emergency department, she was drowsy, disoriented with a Glasgow coma scale of 13/15 and right hemiparesis grade 3/5, with no cranial nerve deficits. She was on treatment for diffuse osteomalacia diagnosed three years ago. Her serum phosphate levels (1.5 mg/dl) were low, calcium levels (9.2 mg/dl) were normal and vitamin D levels (40 nmol/l) were reduced. NEURORADIOLOGYAn urgent CT scan revealed blood in the left medial temporal region that was seen extending to the suprasellar cistern. It was associated with diffuse subarachnoid hemorrhage with intraventricular extension ( Figure 1a). CT angiography was negative for an intracranial aneurysm. The posterior communicating artery and apex of basilar artery were, however, displaced and shifted to the right. An MRI of the brain showed an extra-axial mass in proximity to the dorsum sella causing distortion of the midbrain (Figure 1b T1) with heterogeneous contrast enhancement along with intraparenchymal bleed (Figures 1c, 1d and 1e, T1 with contrast). A skull based meningioma was considered. OPERATIVE FINDINGSAs patient had significant mass effect due to hematoma with features of raised intracranial pressure, she underwent an emergency left temporal craniotomy (navigation guided) with transcortical approach (through middle temporal gyrus) and evacuation of the hematoma. After evacuating the hematoma, tumor was encountered. Tumor was firm in consistency and moderately vascular. It was gradually mobilized after the internal decompression. The posterior portion indenting the brain stem could be mobilized with intact arachnoid between the tumor and brainstem. The medial part of tentorium was visualized and was free from the tumor. Third nerve was noted on the undersurface of tentorium, which was free from the tumor and was preserved. Last portion of the tumor appeared to be adherent to the posterior clinoid and posterior communicating artery. This couldn't be mobilized and was left behind. Postoperatively, patient recovered well. She was alert and conscious with no motor nerve deficits. However, she had third nerve palsy. Postoperative CT scan showed a small hematoma in tumor bed. MICROSCOPIC PATHOLOGYHistologic findings showed a moderately cellular neoplasm composed of round to oval spindle cells that were arranged in sheets. Several variable sized and staghorn vascular channels were scattered throughout the tumor (Figure 1f and 1g). Small amount of normal brain tissue was also included. The individual tumor cells exhibited moderate amount of eosinophilic cytoplasm with indistinct cell borders. Nuclei were hyperchromatic with mild to moderate anisokaryosis (Figure 1h). Additionally, several small to large clusters of mature adipocytes were noted throughout the tumor (Figure 1i). Areas of hemorrhage, focal necrosis and thrombosed blood vessel were also present. There was no significant increase in mitotic activity (Ki-67 labelling index-less than 1%). Immunohistochemical st...

show abstract

Oncogenic osteomalacia from nasal cavity giant cell tumor

Abstract: Clinicians and pathologists must be aware of the clinical symptoms, laboratory abnormalities, and pathologic features of oncogenic osteomalacia, which may be caused by tumors in the head and neck and thus make an exhaustive effort to diagnose the same.

Cited by 16 publications

References 25 publications

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

A 40‐Year‐Old Woman With Intracranial Bleed and Osteomalacia

Contact Info

Product

Resources

About