Case Report
Primary Intramedullary Osteosarcoma of the Maxilla in a Pediatric Patient: A Case Report and Review of the Literature
Ali Amrollahie1*, Peter T Mai2, Jason Portnof1 and Steven I Kaltman1
1Department of Oral and Maxillofacial Surgery, Nova Southeastern University College of Dental Medicine, USA
2Nova Southeastern University College of Dental Medicine, USA
*Corresponding author: Ali Amrollahie, Department of Oral and Maxillofacial Surgery, Nova Southeastern University College of Dental Medicine, Fort Lauderdale, FL. 3200 S University Drive, Ft Lauderdale, FL 33328, USA
Published: 14 Jul, 2018
Cite this article as: Amrollahie A, Mai PT, Portnof J,
Kaltman SI. Primary Intramedullary
Osteosarcoma of the Maxilla in a
Pediatric Patient: A Case Report and
Review of the Literature. Clin Surg.
2018; 3: 2022.
Abstract
Osteosarcomas are the most common malignant neoplasms of bone; mainly found in long bones.
However, osteosarcomas can occur anywhere in the body. Osteosarcomas rarely occur in the head
and neck and are even more uncommon in the maxilla. Primary osteosarcomas of the maxilla in
pediatric patients is extremely uncommon, as head and neck osteosarcomas usually present in the
third to fourth decades of life. Head and neck osteosarcomas usually arise secondary to radiation or
chemotherapy treatment of a preexisting tumor.
In this case report, a lesion was discovered upon routine radiographic and clinical exam in a
pediatric dental office. Once the lesion was biopsied and the well differentiated, intramedullary
osteosarcoma was confirmed histopathologically, a right hemi-maxillectomy was performed.
Virtual surgical planning was based off a medical grade maxillofacial Computed Tomography (CT)
scan. In operable cases, surgical resection of the tumor and surrounding tissue is the gold standard
of treatment. Virtual planning allows for precise and accurate surgical margins. After the patient’s
case was reviewed by the Joe DiMaggio children hospital tumor board, a consensus agreement
on treatment planning deferred post-surgical systemic chemotherapy due to possible long-term
complications.
Introduction
Osteosarcoma is a primary malignant neoplasm arising from mesenchymal stem cells, most commonly found in the long bones around the knee [1,2]. Maxillofacial osteosarcomas are rare and constitute a minor percentage of all diagnosed head and neck tumors [3]. Studies show that the maxilla is less commonly affected than the mandible. Upon occurrence, maxillary osteosarcomas arise more commonly in the alveolar ridge and the maxillary antrum. In contradistinction to osteosarcomas of long bones, which demonstrate a bimodal age distribution, the peak incidence for jaw osteosarcoma is most commonly observed during the 3rd to 4th decades [2,4]. This case study examines the incidence of an osteosarcoma of the maxilla diagnosed in an 8-year-old male and analyzes treatment modalities.
Case Presentation
An 8-year-old African American male presented with his mother to Nova Southeastern University Department of Oral and Maxillofacial Surgery with a history of diffuse swelling above his upper right front teeth consistently for two months. The patient reported that he was asymptomatic with no pain, no numbness, nor any history of fever, loss of appetite, weight loss, epistaxis, nasal obstruction, or change in vision. There was no contributory medical, social or family history. Extraoral examination was unremarkable; no palpable lymph nodes at any level of the head and neck region were clinically detected. Intra-oral examination revealed a firm 3 cm × 3 cm solitary swelling overlaying the buccal aspect of the right maxillary alveolar ridge at the deciduous tooth B, extending anteriorly near to the incisors, but not crossing the midline (Figure 1). There was expansion of the buccal cortices, however no palatal expansion was observed. Orthopantomograph (OPG) revealed a poorly defined mixed-density lesion of the upper right maxilla denoting irregular areas of osteolysis with Garrington’s sign in relation to the upper right impacted canine (Figure 2). Furthermore, a CT scan with intravenous contrast was taken, and axial, coronal and sagittal cuts revealed poorly defined areas of sclerosis with few lytic areas seen involving the right maxillary sinus causing disruption of normal architecture of the involved bone (Figure 3A- 3C). Initially, an incisional biopsy of the right maxillary lesion was performed under general anesthesia. Aspiration was negative for fluid. Perioperatively, the lesion appeared as fibrous tissue with granulation. The initial biopsy revealed osteoid tissue with irregular trabeculae, with osteoblasts appearing large round to oval, epithelioid in appearance with hyperchromatic nucleus and prominent nucleoli and occasional atypical mitoses. Intertrabecular areas showed solid cellular stroma. The tumor exhibited an aggressive infiltrative pattern with diffuse margins. The biopsy was sent to multiple pathologists, who collectively agreed that the lesion represented one of the three differential diagnoses: 1) Desmoplastic fibroma; 2) Well-differentiated intramedullary osteosarcoma; or 3) Atypical fibroma, aggressive in nature. A right hemi-maxillectomy using virtual surgical planning was performed. The lesion was identified using cutting guides and stereolithographic surgical models were fabricated (Figure 4A and 4B). The patient was taken to the operating room for resection of the tumor with at least 1 cm clearance around all the margins (Figure 5A and 5B). The right maxilla was removed in entirety up to the mesial of the first molar. Histopathologic report confirmed the diagnosis of spindle cell neoplasm best classified as well-differentiated low grade intramedullary osteosarcoma (Figure 6A and 6B). The patient was referred to follow up with a pediatric oncologist who recommended observing patient at 3-month intervals.
Figure 1
Figure 1
Intraoral Image showing clinical appearance of solitary lobulated
swelling at the right maxillary alveolar ridge (arrow).
Figure 2
Figure 2
Orthopantomograph (OPG) showing an ill-defined mixed
radiolucent-radiopaque lesion in the right upper jaw (arrow).
Figure 3A
Figure 3A
Axial view of computed tomography demonstrating an expansive,
ill- defined mass centered to the right maxillary alveolar process, destructing
the bone (arrow).
Figure 3B
Figure 3B
Coronal view of computed tomography demonstrating an
expansive, ill- defined mass centered to the right maxillary alveolar process,
destructing the bone (arrow).
Figure 3C
Figure 3C
Sagittal view of computed tomography demonstrating an
expansive, ill- defined mass centered to the right maxillary alveolar process,
destructing the bone (arrow).
Figure 4A
Figure 4A
A right hemi-maxillectomy was planned using Virtual Surgical
Planning (VSP) and the lesion was identified (arrow).
Figure 4B
Figure 4B
The stereolithographic surgical model was fabricated and the
lesion was identified (arrow).
Figure 5A
Figure 5B
Figure 5B
Right hemi-maxillectomy for resection of the tumor with at least 1
cm clearance at all the margins.
Figure 6A
Figure 6A
Histology of well-differentiated osteosarcoma. Photomicrographs
show a degree of cellularity that varies from acellular zones to hypercellular
spindle cells fascicles. Most of the tissue is composed of hypocellular
bundles of very dense collagenous tissue, with small spindle-shaped nuclei,
reminiscent of desmoplastic tumor of bone. (Hematoxylin and Eosin stain,
original magnification × 400).
Discussion
Osteosarcomas are malignant bone tumors characterized
by formation of disorganized immature woven bone or osteoid
tissue from mesenchymal tumor cells [1]. The management of all
variations of osteosarcoma, including low-grade, intramedullary,
and juxtacortical osteosarcomas, are identical with complete surgical
excision of the tumor, in conjunction with wide surgical margins [5].
In cases of osteosarcoma of the jaws, sufficient surgical treatment at
an early stage is a key determinant of favorable outcome. Okinaka
and Takahashi reported a case in which incomplete surgical resection
of maxillary osteosarcoma ultimately led to distant metastasis to
the lungs [6]. The report found that distant metastasis of maxillary
osteosarcoma almost exclusively involved the lungs, and that distant
metastasis presented at a later time than expected given the aggressive
nature of the tumor. Osteosarcomas arising from the maxilla cannot
always be resected with sufficiently broad margins to ameliorate
patient safety [6]. Therefore, surgical therapy is often supplemented
with adjuvant treatment to prevent recurrence. Kupeli et al. [3]
reported a case of pediatric maxillary low-grade osteosarcoma
which was subsequently treated with surgical resection, followed
by chemotherapy. Histopathologic assessment of the resected
tumor revealed positive margins. Due to high risk of recurrence, six
courses of chemotherapy consisting of Cisplatin (120 mg/m²) and
Doxorubicin (30 mg/m²) at 3-week intervals was prescribed. After
chemotherapy treatment was completed he patient showed no signs
of residual or recurrent tumors, and was subsequently in remission
thereafter [3]. Although the use of chemotherapy is controversial it
can be advantageous in its use as adjuvant treatment in cases where
there is a high risk of recurrence; specifically, tumors >4 cm, positive
surgical margins, young age, and previous diagnosis of retinoblastoma
[3]. Consequently, due to the lack of high risk factors in this case,
it was decided that adjuvant chemotherapy was not necessary. The
patient would be closely monitored for any suspicious changes [7].
Furthermore, cases of post-operative radiotherapy have been
cited as possible adjuvant treatment for osteosarcoma of the jaws.
Prabhusankar et al. [8] reported a case of maxillary osteosarcoma,
which approximated the orbital region in a 20-year-old male. The
resected tumor showed positive margins along the superior aspect.
To protect the orbital region during resection, post-operative
radiotherapy was performed [8]. Radiotherapy as adjuvant treatment
is particularly advantageous if surgical resection is incomplete, as
seen in the aforementioned case [8]. Review of the literature revealed
that incidence of osteosarcoma of the maxilla in the first decade is a
rare entity and usually behaves differently from the osteosarcoma of
other bones. These lesions appear to grow slowly and may be observed
after surgical resection [9-12]. A 12-month postoperative imaging of
this case revealed no recurrent lesions (Figure 7A and B). Patient
regularly follows up with a pediatric oncologist every three months.
In summary, children and adolescents of either gender are susceptible
to developing primary non-radiation-associated osteosarcomas in
the head and neck region, most commonly arising in the mandible.
These patients generally present with symptoms of swelling and a
mass of short duration, although dental problems and paresthesia
have also been observed. After radiographic examination, complete
surgical extirpation followed by appropriate adjuvant therapy for
osteosarcoma will allow pediatric patients to demonstrate a better
overall prognosis, irrespective of gender, anatomic location, size of
tumor, or tumor histology. According to Demicco et al. [7] welldifferentiated
gnathic osteosarcoma exhibits a relatively low-grade
malignancy and has an excellent prognosis with wide local excision.
If possible, radiation therapy should be avoided to prevent long-term
growth stunting complications due to the patient’s susceptibility to
growth disturbances in pre-pubescent age [7].
Figure 6B
Figure 6B
Mitotic activity and giant cells are absent, but vital bone spicules
are present at the borders of the specimen. (Hematoxylin and Eosin stain,
original magnification × 400).
Figure 7A
Figure 7A
A 12-month postoperative Magnetic Resonance Imaging (MRI)
of this case shows no mass or abnormal recurrent lesions at anterior maxilla.
Figure 7B
Figure 7B
A 12-month postoperative total body bone scan of this case
shows no evidence of recurrence.
Acknowledgment
The authors thank Dr. Ines Velez, professor and director of Oral and Maxillofacial Pathology, Department of Oral Diagnostic Sciences, Nova Southeastern University College of Dental Medicine and Dr. Robert Ruiz, chief pediatric pathologist at the Joe DiMaggio Children’s Hospital at Memorial for reviewing the histologic data of the patient.
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