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Desmoplastic fibroma of the maxillary sinus
Desmoplastic fibroma of the maxillary sinus TIMOTHY M. CUPERO, MD, RICHARD W. THOMAS, DDS, MD, and SCOTT C. MANNING, MD, Tacoma and Seattle, Washington
Desmoplastic fibromas are locally invasive, benign neoplasms of bone or connective tissue. They rarely metastasize, and they occur primarily in children. These lesions can involve various sites to include the deep tissues of the arm, leg, shoulder, abdominal, and pelvic organs, mediastinum or mesentary.1 Rarely do they arise from the maxillary sinus, with fewer then 40 cases reported in the literature. We present a rare case of a desmoplastic fibroma arising from the maxillary sinus, and discuss its pathophysiology and treatment. CASE REPORT A 14-year-old girl was referred from her dentist after discovering right palatal swelling. On further evaluation the patient was noted to have proptosis, displacement of her right posterior maxillary dentition and swelling of her palate and gingival-buccal sulcus (Fig 1). A CT scan and MRI were performed and demonstrated a 6 × 6 × 3 cm homogenous lesion of the right maxilla extending through the right orbital floor and lateral nasal wall. The tumor completely obliterated the right maxillary sinus and eroded through the right portion of the hard palate (Fig 2). On the basis of clinical and radiographic findings, an aggressive tumor of the maxillary sinus was suspected. Subsequent biopsies confirmed the presence of a desmoplastic fibroma. The patient was taken to the operating room where she underwent a right maxillectomy with orbital preservation via a mid-facial degloving approach. A palatal flap was used to close the resultant defect. Complete excision of the lesion was accomplished with minimal morbidity. To date there has been no evidence of recurrent disease in more than 2 years of patient follow-up.
From the Otolaryngology–Head and Neck Surgery Department (Drs Cupero and Thomas), Madigan Army Medical Center, and the Pediatric Otolaryngology-Head and Neck Surgery Department (Dr Manning), Children’s Hospital and Regional Medical Center. Poster presentation at the 1999 Annual Meeting of the American Association of Otolaryngology–Head and Neck Surgery Foundation, New Orleans, LA, September 1999. Reprint requests: Timothy M. Cupero, MD, Otolaryngology–Head and Neck Surgery, Bassett Army Community Hospital, Ft. Wainwright, AL; e-mail, [email protected]. The opinions or assertions of the authors contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Otolaryngol Head Neck Surg 2001;125:661-2. 0194-5998/2001/$35.00 + 0 23/4/119866 doi:10.1067/mhn.2001.119866
DISCUSSION
Desmoplastic fibromas are locally invasive, benign neoplasms of bone or connective tissue that rarely metastasize. Clinically these lesions occur as painless slowly growing masses. In the early stages patients typically remain asymptomatic until the tumor impinges on adjacent visceral and neurovascular structures. Because of their aggressive growth patterns, desmoids can become life threatening if left untreated. Approximately 23% of extra-abdominal desmoids involve the head and neck region. This number can increase to greater than 30% when considering the incidence in the pediatric population.1 Within the head and neck the most frequently involved site is the neck, followed by the face. Less frequently involved sites include the oral cavity, scalp, sinuses, and orbit.2 A study by Gnepp et al3 reviewed the files of the Armed Forces Institute of Pathology for fibromatosis involving the sinonasal and nasopharyngeal areas from 1885 to 1985. Of the 2 million files reviewed, only 25 cases were discovered. Sixteen patients were male and 9 were female, with ages ranging from 8 months to 62 years. Twenty-two cases involved the maxillary sinus.3 The most common presenting symptoms of fibromatosis of the maxilla included painless swelling or the presence of a mass. Less commonly reported symptoms include loose teeth, recurrent sinusitis, and exophthalmos. Histologic evidence of an infiltrative growth pattern into adjacent soft tissues is typical. Cytologically, tumors are composed of uniform, spindle-shaped fibroblasts in a rich, collagenous matrix with few mitoses.3 The current case demonstrated both these histocytologic findings. The differential diagnosis for desmoids is extensive. Other lesions to consider include nonossifying fibroma, giant cell tumor, chondromyxoid fibroma, and ossifying fibroma. Other important diagnoses to rule out include low-grade fibrosarcoma, reactive fibrosis, and fibrous dysplasia.3 The treatment of choice for desmoids arising in the head and neck is complete surgical excision with a margin of normal tissue. In cases of recurrence or where resection is not feasible for medical or anatomic reasons, radiation and or chemotherapy should be considered. Radiation doses of approximately 60 Gy over 6 to 8 weeks have been reported to control desmoid tumors in a high proportion of patients.4 Goepfert et al5 report661
Otolaryngology– Head and Neck Surgery December 2001
662 CUPERO et al
Fig 1. Gingival-buccal swelling.
Fig 2. MRI depicting extent of tumor.
ed the results of 5 patients treated with postoperative chemotherapy. The tumor completely disappeared in 3 patients and showed significant regression in the other 2. All patients were disease-free at 5 years. Chemotherapy is not without complications, however. Most regimens use adriamycin that is well known for its cardiotoxicity.5 The recurrence rate for this disease process depends on the completeness of the surgical excision. Tumors arising from the neck are far more likely to recur (45%) than those arising from the head (7%).2 Most recurrences recur within the first 2 years, and close follow-up is mandatory. In conclusion, desmoplastic fibromas of the maxillary sinus are rare but aggressive tumors with a propensity for recurrence. Because these are local aggressive
tumors, the treatment of choice is complete surgical excision with close postoperative follow-up. Recurrent tumors or those in which total excision is not possible due to significant morbidity can be treated with radiation or chemotherapy. REFERENCES 1. Enzinger FM, Weiss SW. Soft tissue tumors, 3rd edition. St Louis: CV Mosby; 1995; p. 210-20. 2. Fasching MC, Saleh J, Woods JE. Desmoid tumors of the head and neck. Am J Surg 1988;56:327-30. 3. Gnepp DR, Henley J, Weiss S, Heffner D. Desmoid fibromatosis of the sinonasal tract and nasopharynx: a clinicopathologic study of 25 cases. Cancer 1996;78:2572-9. 4. Kiel KD, Suit HD. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer 1984;54:2051-5. 5. Goepfert H, Cangir A, Ayala AG, et al. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Am J Surg 1982;144:437-44.
Desmoplastic fibromas are locally invasive, benign neoplasms of bone or connective tissue. They rarely metastasize, and they occur primarily in children. These lesions can involve various sites to include the deep tissues of the arm, leg, shoulder, abdominal, and pelvic organs, mediastinum or mesentary.1 Rarely do they arise from the maxillary sinus, with fewer then 40 cases reported in the literature. We present a rare case of a desmoplastic fibroma arising from the maxillary sinus, and discuss its pathophysiology and treatment. CASE REPORT A 14-year-old girl was referred from her dentist after discovering right palatal swelling. On further evaluation the patient was noted to have proptosis, displacement of her right posterior maxillary dentition and swelling of her palate and gingival-buccal sulcus (Fig 1). A CT scan and MRI were performed and demonstrated a 6 × 6 × 3 cm homogenous lesion of the right maxilla extending through the right orbital floor and lateral nasal wall. The tumor completely obliterated the right maxillary sinus and eroded through the right portion of the hard palate (Fig 2). On the basis of clinical and radiographic findings, an aggressive tumor of the maxillary sinus was suspected. Subsequent biopsies confirmed the presence of a desmoplastic fibroma. The patient was taken to the operating room where she underwent a right maxillectomy with orbital preservation via a mid-facial degloving approach. A palatal flap was used to close the resultant defect. Complete excision of the lesion was accomplished with minimal morbidity. To date there has been no evidence of recurrent disease in more than 2 years of patient follow-up.
From the Otolaryngology–Head and Neck Surgery Department (Drs Cupero and Thomas), Madigan Army Medical Center, and the Pediatric Otolaryngology-Head and Neck Surgery Department (Dr Manning), Children’s Hospital and Regional Medical Center. Poster presentation at the 1999 Annual Meeting of the American Association of Otolaryngology–Head and Neck Surgery Foundation, New Orleans, LA, September 1999. Reprint requests: Timothy M. Cupero, MD, Otolaryngology–Head and Neck Surgery, Bassett Army Community Hospital, Ft. Wainwright, AL; e-mail, [email protected]. The opinions or assertions of the authors contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Otolaryngol Head Neck Surg 2001;125:661-2. 0194-5998/2001/$35.00 + 0 23/4/119866 doi:10.1067/mhn.2001.119866
DISCUSSION
Desmoplastic fibromas are locally invasive, benign neoplasms of bone or connective tissue that rarely metastasize. Clinically these lesions occur as painless slowly growing masses. In the early stages patients typically remain asymptomatic until the tumor impinges on adjacent visceral and neurovascular structures. Because of their aggressive growth patterns, desmoids can become life threatening if left untreated. Approximately 23% of extra-abdominal desmoids involve the head and neck region. This number can increase to greater than 30% when considering the incidence in the pediatric population.1 Within the head and neck the most frequently involved site is the neck, followed by the face. Less frequently involved sites include the oral cavity, scalp, sinuses, and orbit.2 A study by Gnepp et al3 reviewed the files of the Armed Forces Institute of Pathology for fibromatosis involving the sinonasal and nasopharyngeal areas from 1885 to 1985. Of the 2 million files reviewed, only 25 cases were discovered. Sixteen patients were male and 9 were female, with ages ranging from 8 months to 62 years. Twenty-two cases involved the maxillary sinus.3 The most common presenting symptoms of fibromatosis of the maxilla included painless swelling or the presence of a mass. Less commonly reported symptoms include loose teeth, recurrent sinusitis, and exophthalmos. Histologic evidence of an infiltrative growth pattern into adjacent soft tissues is typical. Cytologically, tumors are composed of uniform, spindle-shaped fibroblasts in a rich, collagenous matrix with few mitoses.3 The current case demonstrated both these histocytologic findings. The differential diagnosis for desmoids is extensive. Other lesions to consider include nonossifying fibroma, giant cell tumor, chondromyxoid fibroma, and ossifying fibroma. Other important diagnoses to rule out include low-grade fibrosarcoma, reactive fibrosis, and fibrous dysplasia.3 The treatment of choice for desmoids arising in the head and neck is complete surgical excision with a margin of normal tissue. In cases of recurrence or where resection is not feasible for medical or anatomic reasons, radiation and or chemotherapy should be considered. Radiation doses of approximately 60 Gy over 6 to 8 weeks have been reported to control desmoid tumors in a high proportion of patients.4 Goepfert et al5 report661
Otolaryngology– Head and Neck Surgery December 2001
662 CUPERO et al
Fig 1. Gingival-buccal swelling.
Fig 2. MRI depicting extent of tumor.
ed the results of 5 patients treated with postoperative chemotherapy. The tumor completely disappeared in 3 patients and showed significant regression in the other 2. All patients were disease-free at 5 years. Chemotherapy is not without complications, however. Most regimens use adriamycin that is well known for its cardiotoxicity.5 The recurrence rate for this disease process depends on the completeness of the surgical excision. Tumors arising from the neck are far more likely to recur (45%) than those arising from the head (7%).2 Most recurrences recur within the first 2 years, and close follow-up is mandatory. In conclusion, desmoplastic fibromas of the maxillary sinus are rare but aggressive tumors with a propensity for recurrence. Because these are local aggressive
tumors, the treatment of choice is complete surgical excision with close postoperative follow-up. Recurrent tumors or those in which total excision is not possible due to significant morbidity can be treated with radiation or chemotherapy. REFERENCES 1. Enzinger FM, Weiss SW. Soft tissue tumors, 3rd edition. St Louis: CV Mosby; 1995; p. 210-20. 2. Fasching MC, Saleh J, Woods JE. Desmoid tumors of the head and neck. Am J Surg 1988;56:327-30. 3. Gnepp DR, Henley J, Weiss S, Heffner D. Desmoid fibromatosis of the sinonasal tract and nasopharynx: a clinicopathologic study of 25 cases. Cancer 1996;78:2572-9. 4. Kiel KD, Suit HD. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer 1984;54:2051-5. 5. Goepfert H, Cangir A, Ayala AG, et al. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Am J Surg 1982;144:437-44.