Tumor-induced Osteomalacia in a Boy with Maxillary Ossifying Fibroma

Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic disorder of hypophosphatemia associated with elevated tumor-produced fibroblast growth factor 23 (FGF23). Maxillofacial tumors are rarely involved in TIO, especially maxillary TIO in children. We present a 14-year-old boy with osteomalacia and high serum levels of FGF23, a hormone associated with decreased phosphate resorption, due to a maxillary tumor. The patient was treated with oral phosphorus and calcitriol, and surgical removal of the tumor was performed. After 21 months follow-up, he was pain free and had returned to full activity. We review the reported pediatric cases of TIO in the maxillofacial and oral region and discuss the management of these patients considering the published evidence.

Giant peripheral ossifying fibroma with coincidental squamous cell carcinoma: a case report.

BACKGROUND: Peripheral ossifying fibroma is an inflammatory or reactive hyperplasia of the gingiva that is usually small. It is formed by hard tissue in fibrous tissue, and the name "neoplastic lesion" has tended to be used frequently in Europe and America. Clinically, peripheral ossifying fibromas are painless, solitary, exophytic, sessile, or pedunculated and more frequently found in females than in males. To the best of our knowledge, there have been no reports of malignant cases. We herein report the case of giant peripheral ossifying fibroma with squamous cell carcinoma. CASE PRESENTATION: The patient was an 83-year-old Japanese woman who visited our hospital with a gingival massive mass. She was referred to us for an examination and treatment because it was difficult to perform tracheal intubation for surgery of sigmoid colon cancer at another hospital. The mass measured 83 × 58 × 35 mm, and it protruded to the extra-oral region from the right maxillary premolar alveolar region. Panoramic X-ray revealed the shadow of the mass in the right maxillary premolar region, which included some hard tissue. Computed tomography showed scattering calcified images in the mass. Magnetic resonance imaging was not performed because she had vertebral artery clips and screws in her forehead. Given the above findings, we performed a biopsy under local anesthesia. However, we were unable to diagnose absolutely whether the dysplastic squamous epithelia were pseudocarcinomatous hyperplasia of the gingiva or well-differentiated squamous cell carcinoma. Therefore, tumor resection was performed under general anesthesia. The histopathological diagnosis was peripheral ossifying fibroma with coincidental squamous cell carcinoma. There have been no signs of recurrence during follow-up as of 2 years after surgery. CONCLUSIONS: The etiology of giant peripheral ossifying fibroma with squamous cell carcinoma is still not definite. Therefore, careful observation is necessary. It needs to be examined by accumulation of more cases in the future. We herein report the case of giant peripheral ossifying fibroma coincidental squamous cell carcinoma.

Calcifying aponeurotic fibroma around posterior tibialis tendon in an elderly patient with flatfoot: A case report.

RATIONALE: Calcifying aponeurotic fibroma (CAF) is a rare benign fibroblastic tumor that is commonly in the hand or foot of children or adolescents. PATIENT CONCERNS: A 74-year-old female presented with a progressive pain on the medial foot for 3 years ago. The pain aggravated while walking or in a standing position for more than 20 minutes. She also complained of skin contact along the medial aspect of the foot while trying to wear a shoe. DIAGNOSIS: Physical examination revealed a firm, immobile, nontender mass accompanied with flexible flatfoot. On the single heel raise test, loss of the balance and intensification of the pain were observed. Faintly calcified soft tissue mass is shown in plain radiographs without bone involvement. Magnetic resonance imaging revealed a subcutaneous mass with ill-defined circumscribed subcutaneous mass adherent to the thickened PTT. INTERVENTIONS: The patient underwent a complete excisional biopsy, followed by medial displacement calcaneal osteotomy. OUTCOMES: The excised mass was diagnosed to be CAF on the histologic examination. At the 1-year follow-up, patient remained asymptomatic with no evidence of recurrence and all the radiographic parameters demonstrating flat foot improved. LESSONS: This is the first case of CAF located at PTT presenting with both foot pain and functional disability. In this case, complete excision of the causative structure along with alignment correction can contribute to successful postoperative outcome.

Central ossifying fibroma of mandible.

Ossifying fibroma (OF) is a rare, benign, fibro-osseous lesion of the jawbone characterised by replacement of the normal bone with fibrous tissue. The fibrous tissue shows varying amount of calcified structures resembling bone and/or cementum. The central variant of OF is rare, and shows predilection for mandible among the jawbone. Although it is classified as fibro-osseous lesion, it clinically behaves as a benign tumour and can grow to large size, causing bony swelling and facial asymmetry. This paper reports a case of large central OF of mandible in a 40-year-old male patient. The lesion was treated by segmental resection of mandible. Reconstruction of the surgical defect was done using avascular fibula bone graft. Role of three-dimensional printing of jaw and its benefits in surgical planning and reconstruction are also highlighted.

Juvenile Psammomatoid Ossifying Fibroma of the Orbital Roof: A Rare Cause of Proptosis among Children.

INTRODUCTION: Juvenile psammomatoid ossifying fibroma (JPOF) is a rare bone tumor characterized by a predilection for the sinonasal region and a tendency to affect younger patients, with a potential for aggressive growth and high recurrence (30-56%). JPOF warrants complete surgical resection to avoid recurrence. CASE PRESENTATION: In this article, we report a young boy who presented with unilateral prop-tosis with an expansile bony tumor with ground glass appearance involving the left frontal bone and orbital roof on his images. Complete surgical resection was done, and histopathological examination revealed JPOF with abundant psammomatoid bodies. DISCUSSION: This patient is a rare case of neurocranial JOPF and adds new features to the typical features already described for JPOF.

Juvenile psammomatoid ossifying fibroma of the calcaneus.

Juvenile ossifying fibromas (JOFs) are a rare group of fibro-osseous lesions, typically of odontogenic origin. Juvenile psammomatoid ossifying fibroma (JPOF) is one variant, that is, histopathologically distinguished by spherical ossicles resembling psammoma bodies. JPOF tumours are most commonly found in craniofacial skeletal structures and can be locally aggressive. Thus, early management by complete surgical excision is crucial for good outcomes, although recurrence is still possible. Secondary aneurysmal bone cyst (ABC) formation has been reported with JOF lesions, but less commonly with the JPOF variant. We describe an unusual case of JPOF that presented in the calcaneus with secondary ABC formation.

Endoscopic Optic Nerve Decompression for Optic Neuropathy in Sinonasal Fibro-Osseous Tumors.

BACKGROUND: Sinonasal fibro-osseous tumors involving the skull base sometimes result in the encasement of the optic canal and can cause the compressive optic neuropathy. This study aimed to elucidate the optimal timing of endoscopic optic nerve decompression (OND) in cases with optic neuropathy caused by fibro-osseous tumors. METHODS: Medical records were reviewed retrospectively from July 2008 through November 2016. Subjects who underwent surgery with endoscopic OND for optic neuropathy caused by fibro-osseous lesions were enrolled. Pre- and postoperative ophthalmologic evaluation were analyzed, including best-corrected visual acuity, visual field testing, and color vision. RESULTS: A total of 9 patients underwent OND. Seven patients had fibrous dysplasia and 2 patients had juvenile ossifying fibroma. Patients included 6 boys and 3 girls. The average age was 15 years with a range of 8-17 years. Symptom duration ranged from 2 months to 4 years. The mean follow-up period was 28 months (range, 0.8-76.4 months). There was no immediate deteriorated vision after OND. Eight eyes (88.9%) were improved and 1 eye (11.1%) had only visual field improvement. However, patients whose visual impairment was in the range of finger count and hand motion were not recovered beyond the quantitatively measurable level even after OND. CONCLUSIONS: Endoscopic OND in patients with optic neuropathy caused by a fibro-osseous tumor in the sinonasal region is safe and worth trying to improve visual outcomes. Early therapeutic OND is recommended before the patient's visual function is decreased below quantitatively measurable vision.

Deep palmar tumorous conditions of the hand.

PURPOSE: Deep palmar tumors of the hand are very rare, and reported cases are usually benign. The most important issue is frequent anatomical variations with challenging surgical exposure and excision of these lesions. Some case reports or a small series of patients have been reported in the literature. The aim of this study was to present our experience with the deep palmar tumors of the hand. PATIENTS AND METHODS: In the study, retrospective analyses of 43 patients treated with deep palmar tumors of the hand between January 1998 and June 2015 were evaluated. Tumors and tumor-like pathologies of the deep palmar space of the hand were retrospectively evaluated according to age, gender, localization, preoperative symptoms, size, site, treatment methods, histopathology, and early and late complications. Statistics and data analyses were also performed. RESULTS: All 43 pathologies were benign, and histopathologic diagnoses were 10 lipomas, 8 ganglions, 5 giant cell tumor of the tendon sheath, 4 schwannomas, 3 hemangiomas, 3 palmar fibromatosis, 2 epidermal cysts, 2 neurofibroma, 1 angiolymphoid hyperplasia with eosinophilia, 1 granuloma, 1 calcifying aponeurotic fibroma, 1 digital fibroma, 1 foreign body granuloma, and 1 lipofibromatous hamartoma. The most common complication was temporary numbness and paresthesias of the digits. Marginal excision was performed in 40 patients, excision with nerve grafting in 2 patients (with neurofibroma) and carpal tunnel release in one patient with lipofibromatous hamartoma. CONCLUSION: In the deep palmar space of the hand, pathologies are closely associated with tendons, muscles, and neurovascular structures. Preoperative magnetic resonance imaging is helpful for the preoperative diagnosis, evaluating tumor extension, and successful surgical planning. Level of Evidence: IV Therapautic.

Unilateral visual loss resulting from orbital encroachment of an ethmoidal juvenile trabecular ossifying fibroma.

Ossifying fibromas are mainly found in the mandible and maxilla. Reports of them arising in the ethmoid sinuses and orbits are rare. We present a case of an otherwise healthy 20-year-old man with gradual onset of right visual disturbance signified by right relative afferent pupillary defect due to a large unilateral ossifying fibroma arising from the ethmoid sinus compressing the medial half of the right orbit. We emphasise the multidisciplinary combined endoscopic endonasal and external approach to ensure a successful debulking of the fibroma.

Cherubism. A case report / Querubismo. Discusión de un caso

Cherubism is a rare disorder with autosomal dominant inheritance. It is classified as a benign fibro-osseous lesions and may involve either facial bone. Its typical dentofacial deformities are caused by mutations in the SH3BP2 gene. The protein encoded by SH3BP2 had a significant role in the regulation of osteoblasts and osteoclasts. Accordingly with the radiological findings, differential diagnoses includes fibrous dysplasia, giant cell granuloma, osteosarcoma, juvenile ossifying fibroma, fibrous osteoma, odontogenic cyst and hyperparathyroidism. The aim of the present report is twofold. First, we examine the importance of the proper management of these cases. Second, we describe this rare syndrome with the goal of proposing suitable treatments (AU)

El querubismo es una enfermedad rara. Presenta herencia autosómica dominante y es clasificada como una enfermedad fibroósea benigna. Las deformidades típicas de esta dolencia se deben a la alteración del gen SH3BP2 y pueden afectar a cualquier hueso del macizo facial. La proteína codificada por este gen es fundamental para el correcto funcionamiento de osteoblastos y osteoclastos. El diagnóstico diferencial debe incluir: displasia fibrosa, granuloma de células gigantes, osteosarcoma, fibroma osificante juvenil, fibroma osteoide e hiperparatiroidismo (AU)

Large peripheral ossifying fibroma: clinical, histological, and immunohistochemistry aspects. A case report / Gran fibroma osificante periférico: aspectos clínicos, histológicos e inmunohistoquímicos. Presentación de un caso

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Successful treatment of tumour-induced osteomalacia after resection of an oral peripheral ossifying fibroma.

Tumour-induced osteomalacia is a paraneoplastic syndrome wherein bone is affected by a hormone from a tumour that causes renal phosphate wasting and hypophosphataemia. Here, we present the case of a 31-year-old man who has been suffering from generalised bone pains and a spine deformity that led to loss of height. Pertinent findings are low serum phosphorus, low vitamin D and decreased bone mineral density. These findings led to a diagnosis of osteomalacia. However, the finding of an oral mass raised some questions as to what role it plays in the patient's disease. It was suspected that the oral mass (fibroma) was producing a hormone that led to renal phosphate wasting, hypophosphataemia and then osteomalacia. This hypothesis was proven after surgical removal of the mass led to normalisation of the metabolic derangements and eventually led to a resolution of the bone pains.

Orbital ossifying fibroma: A case report. / Fibroma osificante orbitario, a propósito de un caso.

CLINICAL CASE: The case concerns a 34 year-old man, who presented with pain in the medial canthus in his left eye, with proptosis and diplopia. The examination showed exophthalmus and a palpable mass at the inner canthus. The computed tomography revealed a lesion, which was confirmed by biopsy to be a ossifying fibroma. DISCUSSION: Ossifying fibroma is a benign fibro-osseous lesion that mostly affects the jaw. Clinical presentation and complications vary according to its location. Clinical examination, imaging, and histopathology are essential for definitive diagnosis. Surgical treatment is multidisciplinary and long-term follow up is needed.

Fibroma osificante de seno etmoidal con invasión orbitaria: a propósito de un caso / Ossifying fibroma of the ethmoid sinus with orbital invasion: a case report

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¿Cuál es su diagnóstico? / What is your diagnosis?

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Reduction surgery using a combination of a stereolithographic model and navigation system for ossifying fibroma with secondary central giant cell granuloma.

Both central giant cell granuloma (CGCG) and ossifying fibroma (OF) are relatively common diseases. The synchronous presentation of CGCG and OF is, however, an extremely rare occurrence. We present an unusual case with the synchronous presentation of these two diseases in the maxilla and introduce a surgical strategy based on a combination of the stereolithographic model and navigation system for the treatment of gigantic OF with secondary CGCG.

Anetoderma in a patient with terminal osseous dysplasia with pigmentary defects.

Terminal osseous dysplasia with pigmentary defects (TODPD) is a rare, X-linked syndrome classically characterized by distal limb anomalies, pigmented skin defects of the face, and recurrent digital fibromas. X-inactivation plays a major role in determining the range of phenotypic expression. Thus, patients can demonstrate a wide spectrum of disease severity, making accurate diagnosis more challenging. Recent studies have identified a FLNA c.5217G>A mutation as the cause of TODPD, allowing for diagnostic genetic testing. We present a case of molecularly confirmed TODPD in a girl with the 47,XXX chromosomal complement and deformities of the hands and feet, craniofacial abnormalities, and discolored, linear facial lesions. Skin biopsy of the patient's facial lesion revealed absent papillary dermal elastic fibers, consistent with anetoderma, which contrasts with the dermal hypoplasia described in the only other such facial biopsy reported in the literature. The finding of absent elastic fibers in the skin lesions suggests that mutated filamin A, in part, exerts its effects through dysregulated elastin biology, which may explain the nature of many connective tissue pleotropic effects in FLNA-related disorders.

Fibroma osificante juvenil trabecular mandibular: presentación de un caso con reconstrucción inmediata mediante un injerto libre de la cresta ilíaca y costocondral / Juvenile trabecular ossifying fibroma: presentation of a case with immediate costochondral-iliac crest reconstruction

El fibroma osificante juvenil (FOJ) es una lesión fibro-ósea benigna pero con potencial biológico agresivo que afecta al esqueleto craneofacial. Su evolución clínica es rápida, manifestándose principalmente por una asimetría facial de poco tiempo de evolución. Radiográficamente es una lesión radiolúcida uni o multilocular bien definida con contenido radiopaco variable. El tratamiento puede variar dependiendo de las características clínicas, imagenológicas e histopatológicas específicas de cada lesión, sin embargo, un tratamiento agresivo con resección es el más indicado. Se presenta un caso de un niño con extenso FOJ trabecular (FOJT) mandibular. Se realizó la resección completa de la lesión con un margen de seguridad y reconstrucción inmediata con un injerto de la cresta ilíaca y costocondral. Se realizaron controles postoperatorios durante 7 años en los que no se observaron signos de recidiva asociados a una reconstrucción funcional y estética exitosa (AU)

Juvenile ossifying fibroma (JOF) is a benign fibro-osseous lesion with an aggressive biological behavior in most cases. The clinical course is fast, manifesting mainly as a facial asymmetry. Radiographically it presents as a well-defined, uni- or multi-locular radiolucent lesion with variable radiopaque areas. Treatment may vary depending on the clinical, imaging and histopathological specific pattern, but generally an aggressive treatment with resection is the most appropriate. We report a case of a child with an extensive trabecular JOF (TFOJ) of the mandible. We performed a complete resection of the lesion with a security margin and immediate reconstruction with a costochondral-iliac crest bone graft. Postoperative controls were performed for seven years, during which there were no signs of recurrence, demonstrating a successful functional and anesthetic reconstruction (AU)

A peripheral giant cell granuloma with extensive osseous metaplasia or a hybrid peripheral giant cell granuloma-peripheral ossifying fibroma: a case report.

INTRODUCTION: Peripheral giant cell granuloma and peripheral ossifying fibroma are clinicopathologically distinct gingival lesions. Both are included in clinical differential diagnoses of common benign and reactive gingival epulides in humans. It is often impossible to make a clinical distinction between the two entities, thereby making definitive diagnosis dependent on histopathologic features. While our search of the English literature revealed several reports of peripheral giant cell granuloma with 'bone formation', we were unable to identify any reports of hybrid peripheral ossifying fibroma-peripheral giant cell granulomas. CASE PRESENTATION: We report a case of a 44-year-old Caucasian man presenting with a three-month history of swelling of his right posterior mandible, related to an area of previous dental implant restoration. A clinical examination revealed modest extraoral facial swelling of his right posterior mandible, while an intraoral examination showed a 45 × 25 × 15 mm sessile, lobular soft tissue mass of the right posterior mandibular gingiva. The mucosal covering of the lesion exhibited focal surface ulceration. A panoramic radiograph showed two implants at the vicinity of the lesion with no other significant findings. An excisional biopsy of the lesion followed by histopathologic examination of the biopsy specimen revealed salient and distinctive features of peripheral giant cell granuloma and of peripheral ossifying fibroma, estimated at near equal proportions. This raises the possibility of a hybrid odontogenic lesion. CONCLUSION: The presentation of this lesion, with areas of peripheral giant cell granuloma along with a distinct area of extensive osseous formation and stroma reminiscent of a peripheral ossifying fibroma, justifies consideration of this as a possible hybrid lesion. Although the biologic behavior of a combined lesion is not anticipated to deviate significantly from that of either of the single entities, this case resurrects an enduring debate as to whether peripheral giant cell granuloma and peripheral ossifying fibroma are simply parts of a disease spectrum, or whether some of these lesions represent true hybrid lesions. It is therefore recommended that more cases with histopathologic features similar to the lesion in our case be reported in the literature to further elucidate the histogenesis of these lesions.