990
MALIGNANT
gion, and an intact airway. Within a short period, the patient developed significant respiratory distress and metabolic acidosis. Had the intubation been unsuccessful, establishment of a surgical airway would have been indicated. The intraoral stab wound had penetrated the mucosa, submucosa, and buccinator muscle and had lacerated vessels in this area. A search for the exact location of the tear in the facial artery was unsuccessful as the artery and vein were ligated proximal to the level of injury. Ligation of the external carotid artery following injuries to the superficial or deep structures of the face is sometimes indicated. However, this procedure may be only partially effective in controlling bleeding because the anastomoses of right and left arteries across the midline are so numerous.7 Arteriography was considered; however, due to the location of the stab wound it was determined that the treatment of choice was surgical exploration and ligation of bleeding vessels. Summary
OF THE GINGIVA
atively short period. The patient underwent exploration of the wound with ligation of the facial artery and vein. The best method of hemorrhage control is directly at the hemorrhage site. For this reason, we recommend that all penetrating wounds to the face be surgically explored before debridement and closure. References 1. Kruger GO: Oral and Maxillofacial Surgery. St Louis, MO, Mosby, 1984 2. Schwartz SH, Blankenship 29:672, 1971
B-J, Stout RA: J Oral Surg
3. Rahmat H, Amirjamshid A, Kamalian N: Traumatic aneurysm of the facial artery caused by missile injury. J Oral Maxillofac Surg 43:992, 1985 4. DiStefano JF, Mainon W, Mandel MA: False aneurysm of the lingual artery. J Oral Surg 35:918, 1977 5. Akker HP, Lijn F: False aneurysm of the facial artery as a complication of circumferential wiring. Oral Surg 37:514, 1974 6. Bresner M, Brekhe J, et al: False aneurysms region. J Oral Surg 30:307, 1972
A patient with facial lacerations and a patent airway developed major airway compromise in a rel-
J Oral Maxillofac
HEMANGIOPERICYTOMA
7. Dubrul EL: Oral Anatomy. roAmerica, 1988
of the facial
St Louis, MO, Ishiyako Eu-
SurQ
49:990-992,199o
Malignant
Hemangiopericytoma the Gingiva:
of
Report of a Case JOSEPH J. VOGLER,
DMD,* RAO ANDAVOLU,
Hemangiopericytoma is a rare vascular neoplasm first described as an entity by Stout and Murray in 1942.’ They reported a tumor “characterized by the formation of endothelial tubes and sprouts, with a surrounding sheath of rounded and sometimes elongated cells.” These unique cells are found spiraling around the outside of blood vessels and superfiReceived from Lincoln Medical and Mental Health Center, Bronx, NY. * Former Senior Resident, Oral and Maxillofacial Surgery. t Deputy Director of Pathology. $ Chief, Oral and Maxillofacial Surgery. Address correspondence and reprint requests to Dr Leban: Department of Oral and Maxillofacial Surgery, Lincoln Medical and Mental Health Center, 234 E 149th St, Bronx, NY 10451. 0 1990 American geons.
Association
0278-239119014809-0018$3.00/O
of Oral and Maxillofacial
Sur-
MD,t AND STANLEY
G. LEBAN, DMDS
cially resemble leiomyoblasts. Silver connectivetissue staining techniques have demonstrated that they possess long branching processes and, although they do not contain myofibrils, they are capable of contracting, thereby regulating the caliber of the capillary lumen. Its vascular origin accounts for the ubiquitous occurrence of this tumor throughout the body.’ However, although hemangiopericytomas may occur wherever capillaries are found, there is a definite predilection for the musculoskeletal system, with the trunk being the most common site.* Others report occurrence in the brain, urinary bladder, prostate, thyroid gland, and gastrointestinal tract.3-6 Since the original publication of Stout and Murray in 1942, over 400 cases have been reported. Its incidence in the oral cavity is rare. Review of the literature by Das and Gans in t9U7 revealed 9 such
991
VOGLERETAL
cases. Few isolated cases have been reported since that time. Critical review of the literature reveals only one other case of the tumor arising from the gingiva.’ We describe here another case, presenting its clinical features, histologic presentation, and treatment. Report of a Case A 2-year-old boy was first seen in October 1984 at Lincoln Medical and Mental Health Center because of a slowly increasing mass in the left maxillary gingiva, first noticed by his mother several months earlier. The lesion was asymptomatic and the past history was noncontributory. Clinical examination revealed a firm, nodular, nontender 2 x 1 x 0.7-cm mass associated with the left maxillary primary canine. Panoramic and periapical radiographs did not show bony involvement. An incisional biopsy was performed under local anesthesia, and the specimen was submitted to the pathology laboratory with a provisional diagnosis of pyogenic granuloma. A diagnosis of malignant hemangiopericytoma was made following histologic evaluation and outside consultation. In view of the diagnosis, a search for metastases was performed via a complete physical examination: none were found. The patient had surgery in November 1984. Under general anesthesia administered via nasotracheal intubation, a wide local excision of the maxillary alveolus, extending from the midline to the left tuberosity region and including the associated primary teeth and permanent tooth buds, was performed. Primary closure of the residual defect was obtained with a buccal flap. The postoperative course was unremarkable. The margins of excision were free of tumor. The patient was followed for a 9-month period without evidence of local recurrence or metastatic disease. The patient, however, was then lost to further follow-up. PATHOLOGY
The gross specimen was a 2-cm nodular, grayish tan, firm tissue. Histologic study showed a highly vascularized mass composed of bundles of spindleshaped cells with vesicular nuclei showing blunted ends (Fig I). No recognizable storiform pattern characteristic of fibrous histocytoma was seen. The cellular cytoplasm was moderate in amount. Many mitotic figures a few of which were atypical, were seen (Fig 2). No giant cells were seen. The surface of the mass was partly covered by nonkeratinizing squamous mucosa, with an area of ulceration and the presence of acute inflammatory cells. A trichrome stain was inconclusive, as it did not suggest a fibrous or leiomyomatous origin of the tumor. A phosphotungstic acid-hematoxylin (FTAH) stain failed to show the cross-striations of rhabdomyoblasts. Reticulin fibers were seen to surround most tumor cells (Fig 3), delineating them from vascular endothelial cells. The endothelial cells did not show proliferation or atypia. Electron microscopy performed on formalin-fixed tissue failed to show myotibroblasts but did show abun-
FIGURE 1. Photomicrograph showing interlaced bundles of spindle-shaped tumor cells and vascular spaces. (Hematoxylineosin stain. Original magnification, X 100.)
dant intermediate filaments in the cytoplasm. Immunocytochemistry for desmin and vimentin was negative, as it was for factor VIII antigen. Microscopic distinction was made from malignant fibrous histiocytoma and infantile fibrosarcoma because of the lack of characteristic histologic pattern and distinctive reactivity for the fibrous tissue with a trichome stain. A glomus tumor was excluded because of the lack of uniformity and the round shape of the tumor cells with an organoid pattern. Hemangioendothelioma was excluded by negative reactivity to factor VIII antigen and lack of participation by vascular endothelial cells recognized both in routine hematoxylin-eosin and reticulin stains. Vascular leiomyosarcoma was ruled out by failure to demonstrate leiomyoblasts, desmin, and vimentin. With the above features, confirmed by consultation, the tumor was diagnosed as a malignant hemangiopericytoma.
FIGURE 2. Photomicrograph showing tumor cells with moderate amount of cytoplasm, round to oval nuclei, and mitotic figures. (Hematoxylin-eosin stain. Original magnification, X400.)
992
MALIGNANT
HEMANGIOPERICYTOMA
OF THE GINGIVA
Radiation as a primary modality is not indicated due to recurrences following total macroscopic disappearance after doses of 71 Gy.” Summary
FIGURE 3. Reticulin stain showing tumor cells surrounded by reticulin fibers. Note the endothelial cells lining the vessel at lower center. (Reticulin stain. Original magnification, X400.)
Discussion Hemangiopericytoma may occur wherever blood vessels are found. It is a tumor that does not have sufficient arresting gross features to enable it to be recognized clinically.’ It usually presents as a painless slow-growing mass, which is often nodular and well circumscribed. The overlying skin or mucosa is generally normal in appearance and without obvious discoloration. The age distribution varies from neonates to the geriatric population, with a peak appearance in the middle decades. Major studies show an equal sex distribution.’ Holloman et al” in 1971 reported 281 cases of hemangiopericytoma and cited a malignancy rate of 50% for children, somewhat greater than the rate of 41% for the entire series. This tumor is highly malignant, not during the first 5 years, but over a lifetime, with total recurrence, local and distant, of about 50%. It metastasizes via the lymphatits or by hematogenous spread, with the lung being a common site. The treatment of choice is wide local excision. Because of the high incidence of local recurrence, long-term follow-up is advisable. Radiation therapy and chemotherapeutic agents are useful adjuncts, especially following partial excision or as a palliative measure in inoperable cases.
A case of malignant hemangiopericytoma arising in the left maxillary gingiva in a 2-year-old boy is presented. This represents an extremely rare intraoral lesion that is impossible to diagnose clinically. Histologic diagnosis was made by use of special staining techniques, immunohistochemical stains, and electron microscopic evaluation. This tumor shows a high incidence of local recurrence with a poor prognosis. The mainstay of treatment is wide local excision and adequate postoperative follow-up. Acknowledgment The authors thank Dr Louis P. Dehner of the University of Minnesota for his expert opinion, and Dr T.P. Poon of Metropolitan Hospital, New York, NY, for the electronphotomicrographs.
References 1. Stout AP, Murray MR: Hemangiopericytoma. A vascular tumor featuring Zimmerman’s pericytes. Ann Surg 116: 26, 1942 2. O’Brien P, Brasfield RD: Hemangiopericytoma. Cancer 81249, 1962 3. Stout AP: Hemangiopericytoma. A study of twenty five cases. Cancer 2: 1027, 1949 4. Stout AP: Tumors featuring pericytes. Glomus tumor and hemangiopericytoma. Lab Invest 5:217, 1956 5. Reyes JW, Shinozuka H, Garry P, et al: A light and electron microscopic study of hemangiopericytoma of the prostate with local extension. Cancer 40: 1122, 1977 6. Cohen N, Yesner R, Spiro HM: Inflammatory fibroid polyp (hemangiopericytoma of the stomach). Am J Digest Dis 4:549, 1949 7. Das AK, Gans BJ: Hemangiopericytoma of oral cavity. Review of literature and report of age. J Oral Surg 23:456, 1965 8. Piattelli A, Dal Sasso G, Piattelli M: Primary malignant hemangiopericytoma of the gingiva: Report of a case. Acta Stomatol Belg 4:285, 1983 9. Backwenkel KD, Deddans JA: Hemangiopericytoma: Report of a case and comprehensive review of the literature. Cancer 4:8%, 1970 10. Hollman G, Hopner F, Daum R, et al: Beitray Zur Klinek des Hamangiopericytoma. Langebecks Arch Chir 1971 11. Friedman M: Irradiation of hemangiopericytoma of stoat. Radiology 74:721, 1960
MALIGNANT
gion, and an intact airway. Within a short period, the patient developed significant respiratory distress and metabolic acidosis. Had the intubation been unsuccessful, establishment of a surgical airway would have been indicated. The intraoral stab wound had penetrated the mucosa, submucosa, and buccinator muscle and had lacerated vessels in this area. A search for the exact location of the tear in the facial artery was unsuccessful as the artery and vein were ligated proximal to the level of injury. Ligation of the external carotid artery following injuries to the superficial or deep structures of the face is sometimes indicated. However, this procedure may be only partially effective in controlling bleeding because the anastomoses of right and left arteries across the midline are so numerous.7 Arteriography was considered; however, due to the location of the stab wound it was determined that the treatment of choice was surgical exploration and ligation of bleeding vessels. Summary
OF THE GINGIVA
atively short period. The patient underwent exploration of the wound with ligation of the facial artery and vein. The best method of hemorrhage control is directly at the hemorrhage site. For this reason, we recommend that all penetrating wounds to the face be surgically explored before debridement and closure. References 1. Kruger GO: Oral and Maxillofacial Surgery. St Louis, MO, Mosby, 1984 2. Schwartz SH, Blankenship 29:672, 1971
B-J, Stout RA: J Oral Surg
3. Rahmat H, Amirjamshid A, Kamalian N: Traumatic aneurysm of the facial artery caused by missile injury. J Oral Maxillofac Surg 43:992, 1985 4. DiStefano JF, Mainon W, Mandel MA: False aneurysm of the lingual artery. J Oral Surg 35:918, 1977 5. Akker HP, Lijn F: False aneurysm of the facial artery as a complication of circumferential wiring. Oral Surg 37:514, 1974 6. Bresner M, Brekhe J, et al: False aneurysms region. J Oral Surg 30:307, 1972
A patient with facial lacerations and a patent airway developed major airway compromise in a rel-
J Oral Maxillofac
HEMANGIOPERICYTOMA
7. Dubrul EL: Oral Anatomy. roAmerica, 1988
of the facial
St Louis, MO, Ishiyako Eu-
SurQ
49:990-992,199o
Malignant
Hemangiopericytoma the Gingiva:
of
Report of a Case JOSEPH J. VOGLER,
DMD,* RAO ANDAVOLU,
Hemangiopericytoma is a rare vascular neoplasm first described as an entity by Stout and Murray in 1942.’ They reported a tumor “characterized by the formation of endothelial tubes and sprouts, with a surrounding sheath of rounded and sometimes elongated cells.” These unique cells are found spiraling around the outside of blood vessels and superfiReceived from Lincoln Medical and Mental Health Center, Bronx, NY. * Former Senior Resident, Oral and Maxillofacial Surgery. t Deputy Director of Pathology. $ Chief, Oral and Maxillofacial Surgery. Address correspondence and reprint requests to Dr Leban: Department of Oral and Maxillofacial Surgery, Lincoln Medical and Mental Health Center, 234 E 149th St, Bronx, NY 10451. 0 1990 American geons.
Association
0278-239119014809-0018$3.00/O
of Oral and Maxillofacial
Sur-
MD,t AND STANLEY
G. LEBAN, DMDS
cially resemble leiomyoblasts. Silver connectivetissue staining techniques have demonstrated that they possess long branching processes and, although they do not contain myofibrils, they are capable of contracting, thereby regulating the caliber of the capillary lumen. Its vascular origin accounts for the ubiquitous occurrence of this tumor throughout the body.’ However, although hemangiopericytomas may occur wherever capillaries are found, there is a definite predilection for the musculoskeletal system, with the trunk being the most common site.* Others report occurrence in the brain, urinary bladder, prostate, thyroid gland, and gastrointestinal tract.3-6 Since the original publication of Stout and Murray in 1942, over 400 cases have been reported. Its incidence in the oral cavity is rare. Review of the literature by Das and Gans in t9U7 revealed 9 such
991
VOGLERETAL
cases. Few isolated cases have been reported since that time. Critical review of the literature reveals only one other case of the tumor arising from the gingiva.’ We describe here another case, presenting its clinical features, histologic presentation, and treatment. Report of a Case A 2-year-old boy was first seen in October 1984 at Lincoln Medical and Mental Health Center because of a slowly increasing mass in the left maxillary gingiva, first noticed by his mother several months earlier. The lesion was asymptomatic and the past history was noncontributory. Clinical examination revealed a firm, nodular, nontender 2 x 1 x 0.7-cm mass associated with the left maxillary primary canine. Panoramic and periapical radiographs did not show bony involvement. An incisional biopsy was performed under local anesthesia, and the specimen was submitted to the pathology laboratory with a provisional diagnosis of pyogenic granuloma. A diagnosis of malignant hemangiopericytoma was made following histologic evaluation and outside consultation. In view of the diagnosis, a search for metastases was performed via a complete physical examination: none were found. The patient had surgery in November 1984. Under general anesthesia administered via nasotracheal intubation, a wide local excision of the maxillary alveolus, extending from the midline to the left tuberosity region and including the associated primary teeth and permanent tooth buds, was performed. Primary closure of the residual defect was obtained with a buccal flap. The postoperative course was unremarkable. The margins of excision were free of tumor. The patient was followed for a 9-month period without evidence of local recurrence or metastatic disease. The patient, however, was then lost to further follow-up. PATHOLOGY
The gross specimen was a 2-cm nodular, grayish tan, firm tissue. Histologic study showed a highly vascularized mass composed of bundles of spindleshaped cells with vesicular nuclei showing blunted ends (Fig I). No recognizable storiform pattern characteristic of fibrous histocytoma was seen. The cellular cytoplasm was moderate in amount. Many mitotic figures a few of which were atypical, were seen (Fig 2). No giant cells were seen. The surface of the mass was partly covered by nonkeratinizing squamous mucosa, with an area of ulceration and the presence of acute inflammatory cells. A trichrome stain was inconclusive, as it did not suggest a fibrous or leiomyomatous origin of the tumor. A phosphotungstic acid-hematoxylin (FTAH) stain failed to show the cross-striations of rhabdomyoblasts. Reticulin fibers were seen to surround most tumor cells (Fig 3), delineating them from vascular endothelial cells. The endothelial cells did not show proliferation or atypia. Electron microscopy performed on formalin-fixed tissue failed to show myotibroblasts but did show abun-
FIGURE 1. Photomicrograph showing interlaced bundles of spindle-shaped tumor cells and vascular spaces. (Hematoxylineosin stain. Original magnification, X 100.)
dant intermediate filaments in the cytoplasm. Immunocytochemistry for desmin and vimentin was negative, as it was for factor VIII antigen. Microscopic distinction was made from malignant fibrous histiocytoma and infantile fibrosarcoma because of the lack of characteristic histologic pattern and distinctive reactivity for the fibrous tissue with a trichome stain. A glomus tumor was excluded because of the lack of uniformity and the round shape of the tumor cells with an organoid pattern. Hemangioendothelioma was excluded by negative reactivity to factor VIII antigen and lack of participation by vascular endothelial cells recognized both in routine hematoxylin-eosin and reticulin stains. Vascular leiomyosarcoma was ruled out by failure to demonstrate leiomyoblasts, desmin, and vimentin. With the above features, confirmed by consultation, the tumor was diagnosed as a malignant hemangiopericytoma.
FIGURE 2. Photomicrograph showing tumor cells with moderate amount of cytoplasm, round to oval nuclei, and mitotic figures. (Hematoxylin-eosin stain. Original magnification, X400.)
992
MALIGNANT
HEMANGIOPERICYTOMA
OF THE GINGIVA
Radiation as a primary modality is not indicated due to recurrences following total macroscopic disappearance after doses of 71 Gy.” Summary
FIGURE 3. Reticulin stain showing tumor cells surrounded by reticulin fibers. Note the endothelial cells lining the vessel at lower center. (Reticulin stain. Original magnification, X400.)
Discussion Hemangiopericytoma may occur wherever blood vessels are found. It is a tumor that does not have sufficient arresting gross features to enable it to be recognized clinically.’ It usually presents as a painless slow-growing mass, which is often nodular and well circumscribed. The overlying skin or mucosa is generally normal in appearance and without obvious discoloration. The age distribution varies from neonates to the geriatric population, with a peak appearance in the middle decades. Major studies show an equal sex distribution.’ Holloman et al” in 1971 reported 281 cases of hemangiopericytoma and cited a malignancy rate of 50% for children, somewhat greater than the rate of 41% for the entire series. This tumor is highly malignant, not during the first 5 years, but over a lifetime, with total recurrence, local and distant, of about 50%. It metastasizes via the lymphatits or by hematogenous spread, with the lung being a common site. The treatment of choice is wide local excision. Because of the high incidence of local recurrence, long-term follow-up is advisable. Radiation therapy and chemotherapeutic agents are useful adjuncts, especially following partial excision or as a palliative measure in inoperable cases.
A case of malignant hemangiopericytoma arising in the left maxillary gingiva in a 2-year-old boy is presented. This represents an extremely rare intraoral lesion that is impossible to diagnose clinically. Histologic diagnosis was made by use of special staining techniques, immunohistochemical stains, and electron microscopic evaluation. This tumor shows a high incidence of local recurrence with a poor prognosis. The mainstay of treatment is wide local excision and adequate postoperative follow-up. Acknowledgment The authors thank Dr Louis P. Dehner of the University of Minnesota for his expert opinion, and Dr T.P. Poon of Metropolitan Hospital, New York, NY, for the electronphotomicrographs.
References 1. Stout AP, Murray MR: Hemangiopericytoma. A vascular tumor featuring Zimmerman’s pericytes. Ann Surg 116: 26, 1942 2. O’Brien P, Brasfield RD: Hemangiopericytoma. Cancer 81249, 1962 3. Stout AP: Hemangiopericytoma. A study of twenty five cases. Cancer 2: 1027, 1949 4. Stout AP: Tumors featuring pericytes. Glomus tumor and hemangiopericytoma. Lab Invest 5:217, 1956 5. Reyes JW, Shinozuka H, Garry P, et al: A light and electron microscopic study of hemangiopericytoma of the prostate with local extension. Cancer 40: 1122, 1977 6. Cohen N, Yesner R, Spiro HM: Inflammatory fibroid polyp (hemangiopericytoma of the stomach). Am J Digest Dis 4:549, 1949 7. Das AK, Gans BJ: Hemangiopericytoma of oral cavity. Review of literature and report of age. J Oral Surg 23:456, 1965 8. Piattelli A, Dal Sasso G, Piattelli M: Primary malignant hemangiopericytoma of the gingiva: Report of a case. Acta Stomatol Belg 4:285, 1983 9. Backwenkel KD, Deddans JA: Hemangiopericytoma: Report of a case and comprehensive review of the literature. Cancer 4:8%, 1970 10. Hollman G, Hopner F, Daum R, et al: Beitray Zur Klinek des Hamangiopericytoma. Langebecks Arch Chir 1971 11. Friedman M: Irradiation of hemangiopericytoma of stoat. Radiology 74:721, 1960
