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Intranasal glomangioma
Intranasal Glomangioma Robert D. Cullen, BS, and Ehab Y. Hanna, MD Glomangiomas of the nasal cavity are a rare occurrence. Only 9 cases of this vascular tumor have been reported in the literature. A case of intranasal glomangioma is reported with a review of pertinent literature. The histogenesis, clinical presentation, diagnosis, immunohistochemical features, and treatment of these rare tumors are presented. (Am J Otolaryngol 2000;21:402-404. Copyright © 2000 by W.B. Saunders Company) (Editorial Comment: The authors report a novel therapy for an unusual intranasal vascular lesion.)
Glomangiomas are a type of vascular tumor that is rarely found in the upper airway. It is a benign neoplasm that originates from the glomus body, which is a neuromyoarterial structure that helps regulate body temperature by controlling blood flow between capillary beds in the acral regions of the body. It is most commonly found in the extremities, particularly in the fingertips. To our knowledge, there have been only 9 cases reported in the literature of such a tumor occurring in the nasal cavity (Table 1). We present the 10th reported case and a brief review of previously reported cases in the literature. This is also the first case to our knowledge that was treated with laser excision. CASE REPORT Patient 1 is a 50-year-old white woman with a 4-year history of recurrent nosebleeds originating in her right naris. Her first nose bleed occurred approximately 4 years ago. It was packed for several days and subsequently stopped bleeding. Her second incidence of epistaxis occurred several months before her most recent presentation. On experiencing another episode of epistaxis, she was evaluated by her family physician, who noticed a papillomatous mass in her right nasal passage. Her right naris was again packed, and she was referred
From the Department of Otolaryngology, University of Arkansas for Medical Sciences, Little Rock, AR. Address reprint requests to Ehab Y. Hanna, MD, Associate Professor, Department of Otolaryngology— Head and Neck Surgery, University of Arkansas for Medical Sciences, 4301 West Markham, Slot 543, Little Rock, AR 72205. Copyright © 2000 by W.B. Saunders Company 0196-0709/00/2106-0008$10.00/0 doi:10.1053/AJOT.2000.18874 402
to the Department of Otolaryngology at the University of Arkansas for Medical Sciences for further evaluation and management. At that time, the packing was removed and she underwent a thorough head and neck examination. The examination was essentially negative except for the papillomatous mass in her right naris. A head computed tomography showed a 1- ⫻ 2-cm oval mass attached to the inferior turbinate and enhanced with intravenous contrast (Fig 1). The tumor showed no evidence of bony involvement. The mass was removed with Holmium:YAG laser under general anesthesia. Bleeding was controlled with the laser and electrocautery. There were no complications. Histologic examination revealed the mass to be a glomangioma (Figs 3 and 4). Final pathologic examination included positive immunohistochemical staining for muscle-specific actin (Fig 4). Her postoperative course was unremarkable. At 2 years’ follow-up, there is no evidence of recurrence.
DISCUSSION Glomangiomas are benign neoplasms that arise from modified smooth muscle found in the glomus body (glomera). The glomus body is a temperature-sensitive neuromyoarterial structure that regulates arteriolar blood flow in the acral areas of the body. The distribution of glomangiomas parallels that of the glomus body, being most common in the acral areas10 and frequently found beneath the fingernail (thus, the historical name “subungal glomus tumor”). However, the term “glomus tumor” may cause some confusion. In this context, this glomangioma must be differentiated from the paragangliomas of the head and neck, such as the glomus jugulare or glomus tympanicum. These lesions are similar only morphologically in that they are markedly vascular. Glomangiomas are usually less than 1 cm in diameter and are typically elevated, round, red-blue, firm nodules. They are usually exquisitely painful to pressure or cold temperature. In the 9 reported cases of intranasal glo-
American Journal of Otolaryngology, Vol 21, No 6 (November-December), 2000: pp 402-404
INTRANASAL GLOMANGIOMA
TABLE 1.
403
Intranasal Glomangiomas in the Literature
Year Reported
Reference
Age
Sex
1965
Pantazapoulos1
45
F
Inferior turbinate
1972 1974 1979 1984 1986 1992 1993 1997 1999
DeBord2 Fu and Perzin3 Fleury et al4 Potter et al5 Morais6 Alarcos7 Hayes et al8 Arens et al9 Current Case
33 71 24 81 66 55 32 40 50
F F M F M M F M F
Posterior choana Anterior nasal septum Septum Nasal septum Anterior naris Ethmoid sinus and nasal cavity Anterior naris Inferior turbinate Inferior turbinate
mangioma, a consistent constellation of symptoms has not been reported. These tumors have presented with symptoms of obstruction, epistaxis, and/or nasal and facial pain. Three cases were asymptomatic and found on routine physical examination.3,5,6 Histologically, glomangiomas are solid neoplasms composed of clusters of round or polygonal polymorphous glomus cells with large or plump nuclei and scant eosinophilic cytoplasm.11 Smooth muscle components can be shown by immunohistochemical staining for smooth muscle-specific actin.12 Glomangiomas are rarely found in the head and neck. Rarer still is the glomangioma of the nasal cavity. Of 85 cases of vascular tumors of the nasal cavity, nasopharynx, and nasal cavities that Fu and Perzin4 reviewed, only one was glomangioma. This case is the 10th reported case of intranasal glomangioma. Complete excision is usually curative. There is one reported case of recurrent nasal glomangioma, with 6 recurrences that were
Fig 1. Axial and coronal contrasted CT of the paranasal sinuses. A well-circumscribed enhancing mass is seen along the anterior aspect of the right inferior turbinate with no evidence of bony involvement.
Location
Symptoms Nasal and facial pain, obstruction, epistaxis Obstruction Asymptomatic Obstruction Asymptomatic Asymptomatic Obstruction Obstruction Epistaxis Epistaxis
attributed to incomplete excision.8 Malignant glomangiomas (glomangiosarcomas) have been reported. These tumors are locally invasive, may metastasize, and are prone to recur.13 Malignant glomangiomas can be differentiated histologically from their benign counterparts by the presence of mild atypia, spindle-shaped nuclei, various rates of mitotic figures, and the tendency for invasive growth. They have not been reported to occur in the nasal cavity. The Holmium:YAG laser is an appropriate choice for removal of this benign vascular neoplasm. Although technically more difficult to operate than conventional instrumentation, the laser may provide improved hemostasis when dealing with capillary-sized blood vessels.14 Mucosal edema and crust formation have been shown to be more common with the use of the Holmium:YAG laser than with conventional instrumentation in the 1-week postoperative period in endoscopic sinus surgery.15 However, no differences were
404
CULLEN AND HANNA
seen 3 months postoperatively. Minimal intraoperative blood loss occurred in our reported patient. Mild crusting was seen in our patient 1 week after the removal of the tumor. Subsequent healing was unremarkable. CONCLUSION
Fig 2. Histology of glomangioma. Low-power view of glomangioma, showing the marked vascularity of the tumor. The surface mucosa can be seen at the upper portion of the slide. (Hematoxylin & eosin, original magnification ⴛ10.)
Glomangioma of the nasal cavity is a rare occurrence. Patients may present with symptoms of obstruction, pain, or epistaxis. Asymptomatic discovery has been reported as well. Complete excision of these vascular tumors is curative. Laser excision is appropriate and may be superior to conventional methods of removal in the hands of a surgeon experienced in its use. REFERENCES
Fig 3. Histology of glomangioma. Clusters of glomus cells can be seen cuffing the vascular channels of the glomangioma. (Hematoxylin & eosin, original magnification ⴛ40.)
Fig 4. Immunohistochemical stain. Glomus cells react positively when stained for muscle-specific actin (Antimuscle-specific actin, original magnification ⴛ100.)
1. Pantazopoulos PE: Glomus tumor (glomangioma) of the nasal cavity. Arch Otolaryngol 81:83-86, 1965 2. DeBord BA: Unusual presentations in otolaryngology. Surg Clin North Am 52:473-483, 1972 3. Fu YS, Perzin KH: Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A clinicopathologic study: I. General features and vascular tumors. Cancer 33:1275-1288, 1974 4. Fleury P, Bassett JM, Compere JF, et al: Tumours rares de la cloison: Huit cas rapportes. Ann Otolaryngol Chir Cervicofac 96:767-779, 1979 5. Potter AJ, Khatib G, Peppard SB: Intranasal glomus tumor. Arch Otolaryngol 110:755-756, 1984 6. Morais D: Glomangioma or glomus tumor of the nasal vestibulum. An Otorrinolaringol Ibero Am 13:471479, 1986 7. Alarcos L: Tumor glomico de fossa nasal y seno ethmoidal. Acta Otorrinolaringol Esp 43:291-295, 1992 8. Hayes MM, Van der Westhuizen N, Holden GP: Aggressive glomus tumor of the nasal region: Report of a case with multiple local recurrences. Arch Pathol Lab Med 117:649-652, 1993 9. Arens C, Dreyer T, Eistert B: Glomangioma of the nasal cavity. Case reports and literature review. ORL J Otorhinolaryngol 59:179-181, 1997 10. Tsuneyoshi M, Enjoji M: Glomus tumor: A clinicopathologic and electron microscopic study. Cancer 50: 1601-1607, 1982 11. Requena L, Sangueza OP: Cutaneous vascular proliferations. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol 37:887-919, 1997 12. Porter PL, Bigler SA, McNutt M, et al: The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: An immunohistochemical study and review of the literature. Mod Pathol 4:46-52, 1991 13. Hiruta N, Kameda N, Tokudome T, et al: Malignant glomus tumor: A case report and review of the literature. Am J Surg Pathol 21:1096-1103, 1997 14. Gleich LL, Rebeiz EE, Pankratov MM, et al: The Holmium:YAG laser-assisted otolaryngologic procedures. Arch Otolaryngol Head Neck Surg 121:1162-1166, 1995 15. Metson R: Homium:YAG laser endoscopic sinus surgery: A randomized, controlled study. Laryngoscope 106:1-18, 1996 (suppl 77)
Glomangiomas are a type of vascular tumor that is rarely found in the upper airway. It is a benign neoplasm that originates from the glomus body, which is a neuromyoarterial structure that helps regulate body temperature by controlling blood flow between capillary beds in the acral regions of the body. It is most commonly found in the extremities, particularly in the fingertips. To our knowledge, there have been only 9 cases reported in the literature of such a tumor occurring in the nasal cavity (Table 1). We present the 10th reported case and a brief review of previously reported cases in the literature. This is also the first case to our knowledge that was treated with laser excision. CASE REPORT Patient 1 is a 50-year-old white woman with a 4-year history of recurrent nosebleeds originating in her right naris. Her first nose bleed occurred approximately 4 years ago. It was packed for several days and subsequently stopped bleeding. Her second incidence of epistaxis occurred several months before her most recent presentation. On experiencing another episode of epistaxis, she was evaluated by her family physician, who noticed a papillomatous mass in her right nasal passage. Her right naris was again packed, and she was referred
From the Department of Otolaryngology, University of Arkansas for Medical Sciences, Little Rock, AR. Address reprint requests to Ehab Y. Hanna, MD, Associate Professor, Department of Otolaryngology— Head and Neck Surgery, University of Arkansas for Medical Sciences, 4301 West Markham, Slot 543, Little Rock, AR 72205. Copyright © 2000 by W.B. Saunders Company 0196-0709/00/2106-0008$10.00/0 doi:10.1053/AJOT.2000.18874 402
to the Department of Otolaryngology at the University of Arkansas for Medical Sciences for further evaluation and management. At that time, the packing was removed and she underwent a thorough head and neck examination. The examination was essentially negative except for the papillomatous mass in her right naris. A head computed tomography showed a 1- ⫻ 2-cm oval mass attached to the inferior turbinate and enhanced with intravenous contrast (Fig 1). The tumor showed no evidence of bony involvement. The mass was removed with Holmium:YAG laser under general anesthesia. Bleeding was controlled with the laser and electrocautery. There were no complications. Histologic examination revealed the mass to be a glomangioma (Figs 3 and 4). Final pathologic examination included positive immunohistochemical staining for muscle-specific actin (Fig 4). Her postoperative course was unremarkable. At 2 years’ follow-up, there is no evidence of recurrence.
DISCUSSION Glomangiomas are benign neoplasms that arise from modified smooth muscle found in the glomus body (glomera). The glomus body is a temperature-sensitive neuromyoarterial structure that regulates arteriolar blood flow in the acral areas of the body. The distribution of glomangiomas parallels that of the glomus body, being most common in the acral areas10 and frequently found beneath the fingernail (thus, the historical name “subungal glomus tumor”). However, the term “glomus tumor” may cause some confusion. In this context, this glomangioma must be differentiated from the paragangliomas of the head and neck, such as the glomus jugulare or glomus tympanicum. These lesions are similar only morphologically in that they are markedly vascular. Glomangiomas are usually less than 1 cm in diameter and are typically elevated, round, red-blue, firm nodules. They are usually exquisitely painful to pressure or cold temperature. In the 9 reported cases of intranasal glo-
American Journal of Otolaryngology, Vol 21, No 6 (November-December), 2000: pp 402-404
INTRANASAL GLOMANGIOMA
TABLE 1.
403
Intranasal Glomangiomas in the Literature
Year Reported
Reference
Age
Sex
1965
Pantazapoulos1
45
F
Inferior turbinate
1972 1974 1979 1984 1986 1992 1993 1997 1999
DeBord2 Fu and Perzin3 Fleury et al4 Potter et al5 Morais6 Alarcos7 Hayes et al8 Arens et al9 Current Case
33 71 24 81 66 55 32 40 50
F F M F M M F M F
Posterior choana Anterior nasal septum Septum Nasal septum Anterior naris Ethmoid sinus and nasal cavity Anterior naris Inferior turbinate Inferior turbinate
mangioma, a consistent constellation of symptoms has not been reported. These tumors have presented with symptoms of obstruction, epistaxis, and/or nasal and facial pain. Three cases were asymptomatic and found on routine physical examination.3,5,6 Histologically, glomangiomas are solid neoplasms composed of clusters of round or polygonal polymorphous glomus cells with large or plump nuclei and scant eosinophilic cytoplasm.11 Smooth muscle components can be shown by immunohistochemical staining for smooth muscle-specific actin.12 Glomangiomas are rarely found in the head and neck. Rarer still is the glomangioma of the nasal cavity. Of 85 cases of vascular tumors of the nasal cavity, nasopharynx, and nasal cavities that Fu and Perzin4 reviewed, only one was glomangioma. This case is the 10th reported case of intranasal glomangioma. Complete excision is usually curative. There is one reported case of recurrent nasal glomangioma, with 6 recurrences that were
Fig 1. Axial and coronal contrasted CT of the paranasal sinuses. A well-circumscribed enhancing mass is seen along the anterior aspect of the right inferior turbinate with no evidence of bony involvement.
Location
Symptoms Nasal and facial pain, obstruction, epistaxis Obstruction Asymptomatic Obstruction Asymptomatic Asymptomatic Obstruction Obstruction Epistaxis Epistaxis
attributed to incomplete excision.8 Malignant glomangiomas (glomangiosarcomas) have been reported. These tumors are locally invasive, may metastasize, and are prone to recur.13 Malignant glomangiomas can be differentiated histologically from their benign counterparts by the presence of mild atypia, spindle-shaped nuclei, various rates of mitotic figures, and the tendency for invasive growth. They have not been reported to occur in the nasal cavity. The Holmium:YAG laser is an appropriate choice for removal of this benign vascular neoplasm. Although technically more difficult to operate than conventional instrumentation, the laser may provide improved hemostasis when dealing with capillary-sized blood vessels.14 Mucosal edema and crust formation have been shown to be more common with the use of the Holmium:YAG laser than with conventional instrumentation in the 1-week postoperative period in endoscopic sinus surgery.15 However, no differences were
404
CULLEN AND HANNA
seen 3 months postoperatively. Minimal intraoperative blood loss occurred in our reported patient. Mild crusting was seen in our patient 1 week after the removal of the tumor. Subsequent healing was unremarkable. CONCLUSION
Fig 2. Histology of glomangioma. Low-power view of glomangioma, showing the marked vascularity of the tumor. The surface mucosa can be seen at the upper portion of the slide. (Hematoxylin & eosin, original magnification ⴛ10.)
Glomangioma of the nasal cavity is a rare occurrence. Patients may present with symptoms of obstruction, pain, or epistaxis. Asymptomatic discovery has been reported as well. Complete excision of these vascular tumors is curative. Laser excision is appropriate and may be superior to conventional methods of removal in the hands of a surgeon experienced in its use. REFERENCES
Fig 3. Histology of glomangioma. Clusters of glomus cells can be seen cuffing the vascular channels of the glomangioma. (Hematoxylin & eosin, original magnification ⴛ40.)
Fig 4. Immunohistochemical stain. Glomus cells react positively when stained for muscle-specific actin (Antimuscle-specific actin, original magnification ⴛ100.)
1. Pantazopoulos PE: Glomus tumor (glomangioma) of the nasal cavity. Arch Otolaryngol 81:83-86, 1965 2. DeBord BA: Unusual presentations in otolaryngology. Surg Clin North Am 52:473-483, 1972 3. Fu YS, Perzin KH: Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A clinicopathologic study: I. General features and vascular tumors. Cancer 33:1275-1288, 1974 4. Fleury P, Bassett JM, Compere JF, et al: Tumours rares de la cloison: Huit cas rapportes. Ann Otolaryngol Chir Cervicofac 96:767-779, 1979 5. Potter AJ, Khatib G, Peppard SB: Intranasal glomus tumor. Arch Otolaryngol 110:755-756, 1984 6. Morais D: Glomangioma or glomus tumor of the nasal vestibulum. An Otorrinolaringol Ibero Am 13:471479, 1986 7. Alarcos L: Tumor glomico de fossa nasal y seno ethmoidal. Acta Otorrinolaringol Esp 43:291-295, 1992 8. Hayes MM, Van der Westhuizen N, Holden GP: Aggressive glomus tumor of the nasal region: Report of a case with multiple local recurrences. Arch Pathol Lab Med 117:649-652, 1993 9. Arens C, Dreyer T, Eistert B: Glomangioma of the nasal cavity. Case reports and literature review. ORL J Otorhinolaryngol 59:179-181, 1997 10. Tsuneyoshi M, Enjoji M: Glomus tumor: A clinicopathologic and electron microscopic study. Cancer 50: 1601-1607, 1982 11. Requena L, Sangueza OP: Cutaneous vascular proliferations. Part II. Hyperplasias and benign neoplasms. J Am Acad Dermatol 37:887-919, 1997 12. Porter PL, Bigler SA, McNutt M, et al: The immunophenotype of hemangiopericytomas and glomus tumors, with special reference to muscle protein expression: An immunohistochemical study and review of the literature. Mod Pathol 4:46-52, 1991 13. Hiruta N, Kameda N, Tokudome T, et al: Malignant glomus tumor: A case report and review of the literature. Am J Surg Pathol 21:1096-1103, 1997 14. Gleich LL, Rebeiz EE, Pankratov MM, et al: The Holmium:YAG laser-assisted otolaryngologic procedures. Arch Otolaryngol Head Neck Surg 121:1162-1166, 1995 15. Metson R: Homium:YAG laser endoscopic sinus surgery: A randomized, controlled study. Laryngoscope 106:1-18, 1996 (suppl 77)