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Revista Española de Cirugía Oral y Maxilofacial

versión On-line ISSN 2173-9161versión impresa ISSN 1130-0558

Rev Esp Cirug Oral y Maxilofac vol.30 no.5 Barcelona sep./oct. 2008

 

PÁGINA DEL RESIDENTE

 

What is the diagnosis and how should it be treated?

¿Cuál es el diagnóstico y el tratamiento?

 

 

A 53-year-old man was being followed up by his primary care physician for left nasal obstruction of several months duration that was resistant to topical treatment. He presented homolateral exophthalmia in recent weeks. CT disclosed an expansive mass in the left maxillary sinus, nasal fossas, left frontal sinus, and sphenoidal sinus that encroached on the orbit. The patient was referred to emergency maxillofacial surgery.

The patient’s personal history included cardias incompetence, spontaneous pneumothorax 30 years earlier, and an occupational history of working in a setting with aromatic hydrocarbons and carpentry.

He presented left exophthalmia and left nasal respiratory insufficiency in the physical examination. A tumor was visible in the left nasal fossa on direct rhinoscopy. An attempt to obtain a biopsy of the tumor under local anesthesia was impeded by profuse bleeding. Magnetic resonance imaging was ordered in preparation for tumor resection under general anesthesia.

MRI result: tumor mass with infiltration of the nasal septum, sphenoidal sinus, frontal sinus, maxillary sinus, and left orbit medial wall. Orbital infiltration of the intra and extraconal fat caused displacement of the medial rectus muscle and left inferior rectus muscle. The mass contacted the optic nerve. Invasion of the sphenoidal sinus was in contact with both carotids and the anterior wall of the sella turcica (Fig. 1).

 


Naso-maxillo-orbital osteogenic sarcoma. Transcranial approach

Sarcoma osteogénico naso-maxilo-orbitario. Abordaje transcraneal

 

 

L. Pingarrón Martín1, J.L. del Castillo Pardo de Vera2, E. González Obeso3, E. Palacios Weiss1, J.L. Cebrián Carretero2, F. Lopez-Barea4, M. Burgueño García5

1 Médico Residente. Servicio de Cirugía Oral y Maxilofacial.
2 Médico Adjunto. Servicio de Cirugía Oral y Maxilofacial.
3 Médico Residente. Servicio de Anatomía Patológica.
4 Médico Adjunto. Servicio de Anatomía Patológica.
5 Jefe de Servicio. Servicio de Cirugía Oral y Maxilofacial.
Hospital Universitario La Paz, Madrid. España

Correspondence

 

 

Intraoperative biopsy performed under general anesthesia and through a left lateral rhinotomy incision confirmed the presence of sarcoma. Access to the tumor was obtained by a bicoronal approach, frontal craniotomy and a bilateral supraorbital bar (Fig. 2). Medial ethmoidal maxillectomy with orbital exenteration and reconstruction with temporal muscle flap was performed. Bone infiltration of the skull base (lamina cribosa and crista galli) was not evident.

Histopathologic study of the surgical piece revealed a malignant mesenchymal tumor of high cellular density arrayed in sheets and adopting different patterns in which fusiform, epithelioid, highly collagenized, and myxoid areas alternated. No hemangiopericytoid vascular pattern was observed. Cells had scant cytoplasm or abundant eosinophilic cytoplasm (epithelioid cells); nuclei were elongated or vesicular, with evident anisokaryosis. The mitotic activity was regionally high (more than 10 high magnification fields, including atypical mitoses). The interstice showed differentiation of osteoid trabeculae and bone in intimate contact with the tumor tissue; despite osteoblastic margins on the periphery of the trabeculae. Microscopic nests of atypical hyaline cartilage with necrotic tumor foci were recognized.

Immunohistochemistry showed tumor cells with extensive and diffuse positivity to muscular actin (HHF 35) and negativity to desmin and osteocalcin. The proliferation index (Ki 67) was 20%.

Histopathologic diagnosis of the surgical piece: predominantly fibroblastic osteosarcoma of the maxillary sinus, Broders grade III-IV.

 

Discussion

Osteogenic sarcoma, or osteosarcoma, is the most frequent primary bone tumor (17 to 21%) in people younger than 40 years, affecting the mandible and maxilla in approximately 6.5% of cases. Its frequency is estimated at 0.07- 0.4/100000 inhabitants.

The preferred location of the tumor in the human economy is the long bone, with predilection for the distal femoral metaphysis, proximal tibia, and humeral metaphysis. The mortality associated with osteosarcoma in these locations is high, with a 5-year survival of 20%. The bibliography reviewed did not indicate any relation between tumor location and gender.

Of the several osteosarcoma classifications that exist, the one most used is the following:

•Conventional osteosarcoma: Conventional sarcoma occurs mainly in long bone metaphyses of young people and it usually is high grade. Four categories are defined: osteoblastic, chondroblastic, fibroblastic, and mixed.

• Maxillary osteosarcoma: Maxillary osteosarcoma usually appears 1 or 2 decades later than conventional osteosarcoma and is of low or moderate grade. Chondroblastic type osteosarcoma is present in around 50%.

These tumors have a variety of clinical manifestations; presentation as a rapidly growing, painless, or mildly painful bone tumor is a constant finding in published cases. Edema and local paresthesia are infrequent symptoms and seem to be associated with rapid tumor growth, rather than distinctive findings of osteosarcoma.

Personal history predisposing to osteogenic sarcoma: Paget’s bone disease and irradiation.

Sarcomas of the nasal cavity and paranasal sinuses are rare and are responsible for 7 to 28% of all head and neck sarcomas. They represent 0.5-1% of all tumors in the nasal fossa and paranasal sinuses. In the maxilla and mandible, presentation of osteosarcoma at later ages (after the age of 40 years) and a higher survival rate (75% mean survival with a disease-free interval of 8 years) differentiate it from osteosarcoma in other locations. In this location, osteosarcomas have a high percentage of local recurrence and a low risk of metastatic spread, generally causing the death of the patient by intracranial propagation or uncontrolled local invasion. The tumor location correlates directly with prognosis.

Among the paranasal sinuses, the maxillary sinus is the most frequent location, followed by the ethmoidal sinuses and frontal sinus. In the face, the mandibular location is more common than the maxilla. Osteosarcomas of the paranasal sinuses appear in patients over 30 and show no gender predilection.

The preoperative clinical diagnosis includes the differential diagnosis with other conditions like granuloma, epidermoid carcinoma, exostosis, and chondrosarcoma. The definitive diagnosis is provided by histopathologic study of the biopsy material.

Radiographic interpretation of these tumors is difficult because there are no radiographic findings defined as typical in the literature. We have to rely more on the general criteria of malignancy than on specific tumor criteria when assessing the radiographic findings. The presence of a destructive unifocal lesion with poorly defined margins and a predominantly sclerotic, lytic, or mixed pattern suggests the possibility of osteogenic sarcoma. The classification of osteosarcoma according to the Broders Index is based on 4 grades of malignancy depending on the density and degree of atypia of tumor cells (minimum, discrete, moderate, and high) (Figs. 3 and 4).

The therapy of choice is surgical, alone or in combination with radiotherapy, and the survival rate is as high as 85%. The radiotherapy dose used as adjuvant treatment, never in monotherapy, is usually 6000 rad. The recommended treatment of head and neck osteosarcoma is surgical resection associated with radiotherapy and/or chemotherapy in the majority of cases, depending on the degree of differentiation and cell line.

Independently of the therapeutic modality chosen, the importance of long term follow-up of these tumors must be emphasized because patient life expectancy is limited by local recurrence and infiltration of the skull base.

 

 

Correspondence:
Lorena Pingarrón Martín
Servicio de Cirugía Oral y Maxilofacial
Hospital Universitario La Paz
Pº Castellana 261. 28046 Madrid. España
Email: lorenapingarron@yahoo.es

 

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