CASE REPORTS PAUL A. LEVINE, MD Ca.e Report Editor
Myxomas of the maxilla in infants and children DAVID K. HAYES, MAJ. MC. JAMES M. MADSEN. CPT. MC, ROGER SIMPSON, MAJ. MC. and ROBERT C. JARCHOW, COL, MC, Ft. Sam Houston, Texas
Osseous myxomas are slow-growing, locally invasive tumors that are found exclusively in the facial skeleton and most frequently manifest as a slow-growing palatal or facial swelling. Patients may also manifest malocclusion, loose teeth, or paresthesias of the fifth cranial nerve. An increased rate of growth has been noted by many authors after local trauma, (e.g., tooth extraction or blunt injury, as in the case presented). J The frequent involvement of teeth has lead to speculation that these tumors are odontogenic in origin.' The patients are otherwise asymptomatic, and children may seem unaware of the tumor until the parent notices an external facial mass and seeks medical advice. The behavior of osseous myxomas, like their histologic appearance, is distinctly benign and we found no reports of metastatic spread or death caused by an osseous myxoma in the literature. The preoperative evaluation of infants or children may be difficult and diagnosis of these tumors may not be made until surgical exploration has been done. A dilemma arises when the surgeon is confronted with a histologically benign-appearing tumor in the mid-face of an infant or young child. Inadequate surgical excision is believed to be responsible for the reported 25% to 30% recurrence rate for osseous myxomas. 3 This is a report of the presentation of an osseous myxoma of the midfacial skeleton in a 12-month old girl and a summarization of the 20 cases we found in reviewing the current English literature (Table 1). Fig. 1. Twelve-month old girl with tumor obliterating the right nasolabial crease.
From Brooke Army Medical Center. The opinions or assertions contained herein are the private views of the authors and are not to be construed as reflecting the views of the Department of the Army or the Department of Defense. Submitted for publication Feb. 26, 1990; revision received May 12. 1991; accepted May 21,1991. Reprint requests: David K. Hayes, MAJ, MD, Otolaryngology Service, Walter Reed Army Medical Center. Washington, DC203075001. 23/4/31511
464
CASE REPORT The patient was a 12-month old girl who was in good health until 3 months before admission, when her parents first noticed a nickel-sized mass just lateral to her right nares. The mass increased in size during the ensuing 3 months and the parents took her to their pediatrician for evaluation. The child had no noticeable discomfort from the mass, there was no sign of inflammation, and the child appeared to be in good
Volume 105 Number 3 September 1991
Case Reports 465
Fig. 2. T2-weighted parasagittal MR scan demonstrates the infraorbital pseudoencapsulated mass In the anterior wall of the maxillary sinus.
health. In reviewing her medical history, the parents remembered that she fell and hit her nose on a table, with an episode of self-limited epistaxis, a few weeks before they first noticed the mass. Examination revealed a 3- x 2-cm rock-hard nontender right facial mass, obliterating the right nasolabial crease (Fig. I). The right nares was patent, with slight medial displacement of the inferior turbinate. Dentition was normal for the patient's age andthe cranial nerves appeared fully intact, although the patient was too young for an accurate assessment of V2 to be obtained. An axial CT scan demonstrated a lateral nasal soft tissue mass originating from the right maxilla. The anterior wall of the maxillary sinus and the inferior turbinate were displaced, with no evidence of bone destruction. A T2-weighted MRI demonstrated a homogeneous subperiosteal mass of the right anterior maxillary sinus wall with an indistinct capsule (Fig. 2). Preoperative differential diagnosis included odontogenic or
aneurysmal bone cyst, fibrous dysplasia, eosinophilic granuloma, chondrosarcoma, ossifying fibroma, osteosarcoma, and neuroblastoma. A fine-needle aspirate of the mass was performed and nasal glioma and melanotic neuroectodermal tumor of infancy were added to the differential diagnosis. Exposure through a sublabial approach revealed a subperiosteal soft tissue paranasal mass with an external bony shell. The anterior maxillary wall was displaced posteriorly, with loss of distinct tumor margins and invasion of the right inferior turbinate requiring a mid-facial degloving procedure and excision of the turbinate. Roots of two teeth were in contact with the tumor. Grossly, the tumor was a firm, mucoid, hypovascular mass that appeared to be encapsulated. Histologic examination demonstrated the characteristic picture for this tumor: uniformly small spindle-shaped cells in a myxoid stroma, with rare mitoses and inconspicuous vascularity (Fig. 3). The postoperative course was unremarkable and there was no evidence of recurrence at a 3-month clinic examination.
466
OtolaryngologvHead and Neck Surgery
Case Reports
,
Fig. 3. low-power photomicrograph. Spindle-shaped cells in a myxoid stroma.
DISCUSSION Myxomas were first described by Virchow in 1871.4 He noted their resemblance to the mucinous substance he observed in umbilical cords and remarked that these tumors were a separate entity that did not differentiate into other types. Very little further work is reported in the literature until the late 1940's, when Stout" redefined the histologic criteria for diagnosing myxomas: benign mesenchymal tumors composed of stellate or spindleshaped cells in a loose myxomatous stroma. Thoma and Goldman? first separated myxomas of the maxilla and mandible from other myxomas in 1947. Their findings were as follows: average age, 30 years; occurrence,
Myxomas of the maxilla in infants and children DAVID K. HAYES, MAJ. MC. JAMES M. MADSEN. CPT. MC, ROGER SIMPSON, MAJ. MC. and ROBERT C. JARCHOW, COL, MC, Ft. Sam Houston, Texas
Osseous myxomas are slow-growing, locally invasive tumors that are found exclusively in the facial skeleton and most frequently manifest as a slow-growing palatal or facial swelling. Patients may also manifest malocclusion, loose teeth, or paresthesias of the fifth cranial nerve. An increased rate of growth has been noted by many authors after local trauma, (e.g., tooth extraction or blunt injury, as in the case presented). J The frequent involvement of teeth has lead to speculation that these tumors are odontogenic in origin.' The patients are otherwise asymptomatic, and children may seem unaware of the tumor until the parent notices an external facial mass and seeks medical advice. The behavior of osseous myxomas, like their histologic appearance, is distinctly benign and we found no reports of metastatic spread or death caused by an osseous myxoma in the literature. The preoperative evaluation of infants or children may be difficult and diagnosis of these tumors may not be made until surgical exploration has been done. A dilemma arises when the surgeon is confronted with a histologically benign-appearing tumor in the mid-face of an infant or young child. Inadequate surgical excision is believed to be responsible for the reported 25% to 30% recurrence rate for osseous myxomas. 3 This is a report of the presentation of an osseous myxoma of the midfacial skeleton in a 12-month old girl and a summarization of the 20 cases we found in reviewing the current English literature (Table 1). Fig. 1. Twelve-month old girl with tumor obliterating the right nasolabial crease.
From Brooke Army Medical Center. The opinions or assertions contained herein are the private views of the authors and are not to be construed as reflecting the views of the Department of the Army or the Department of Defense. Submitted for publication Feb. 26, 1990; revision received May 12. 1991; accepted May 21,1991. Reprint requests: David K. Hayes, MAJ, MD, Otolaryngology Service, Walter Reed Army Medical Center. Washington, DC203075001. 23/4/31511
464
CASE REPORT The patient was a 12-month old girl who was in good health until 3 months before admission, when her parents first noticed a nickel-sized mass just lateral to her right nares. The mass increased in size during the ensuing 3 months and the parents took her to their pediatrician for evaluation. The child had no noticeable discomfort from the mass, there was no sign of inflammation, and the child appeared to be in good
Volume 105 Number 3 September 1991
Case Reports 465
Fig. 2. T2-weighted parasagittal MR scan demonstrates the infraorbital pseudoencapsulated mass In the anterior wall of the maxillary sinus.
health. In reviewing her medical history, the parents remembered that she fell and hit her nose on a table, with an episode of self-limited epistaxis, a few weeks before they first noticed the mass. Examination revealed a 3- x 2-cm rock-hard nontender right facial mass, obliterating the right nasolabial crease (Fig. I). The right nares was patent, with slight medial displacement of the inferior turbinate. Dentition was normal for the patient's age andthe cranial nerves appeared fully intact, although the patient was too young for an accurate assessment of V2 to be obtained. An axial CT scan demonstrated a lateral nasal soft tissue mass originating from the right maxilla. The anterior wall of the maxillary sinus and the inferior turbinate were displaced, with no evidence of bone destruction. A T2-weighted MRI demonstrated a homogeneous subperiosteal mass of the right anterior maxillary sinus wall with an indistinct capsule (Fig. 2). Preoperative differential diagnosis included odontogenic or
aneurysmal bone cyst, fibrous dysplasia, eosinophilic granuloma, chondrosarcoma, ossifying fibroma, osteosarcoma, and neuroblastoma. A fine-needle aspirate of the mass was performed and nasal glioma and melanotic neuroectodermal tumor of infancy were added to the differential diagnosis. Exposure through a sublabial approach revealed a subperiosteal soft tissue paranasal mass with an external bony shell. The anterior maxillary wall was displaced posteriorly, with loss of distinct tumor margins and invasion of the right inferior turbinate requiring a mid-facial degloving procedure and excision of the turbinate. Roots of two teeth were in contact with the tumor. Grossly, the tumor was a firm, mucoid, hypovascular mass that appeared to be encapsulated. Histologic examination demonstrated the characteristic picture for this tumor: uniformly small spindle-shaped cells in a myxoid stroma, with rare mitoses and inconspicuous vascularity (Fig. 3). The postoperative course was unremarkable and there was no evidence of recurrence at a 3-month clinic examination.
466
OtolaryngologvHead and Neck Surgery
Case Reports
,
Fig. 3. low-power photomicrograph. Spindle-shaped cells in a myxoid stroma.
DISCUSSION Myxomas were first described by Virchow in 1871.4 He noted their resemblance to the mucinous substance he observed in umbilical cords and remarked that these tumors were a separate entity that did not differentiate into other types. Very little further work is reported in the literature until the late 1940's, when Stout" redefined the histologic criteria for diagnosing myxomas: benign mesenchymal tumors composed of stellate or spindleshaped cells in a loose myxomatous stroma. Thoma and Goldman? first separated myxomas of the maxilla and mandible from other myxomas in 1947. Their findings were as follows: average age, 30 years; occurrence,
