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Endoscopic treatment of ethmoidal hemangioendothelioma: case report and review of the literature
American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287 – 290 www.elsevier.com/locate/amjoto
Endoscopic treatment of ethmoidal hemangioendothelioma: case report and review of the literature Lucia Semino, MDa,4, Fabio Pagella, MDa, Giovanni Delu`, MDb, Alessandra Todeschini, MDc, Ombretta Luinetti, MDd, Federico Zappoli, MDe, Paolo Castelnuovo, MDb a ENT Department, University of Pavia, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy ENT Department, University of Insubria, Ospedale di Circolo, Fondazione Macchi, 21100 Varese, Italy c Neuroradiology Department, Istituto Neurologico IRCCS Fondazione C. Mondino, 27100 Pavia, Italy d Department of Human Pathology, University of Pavia, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy e Department of Radiology, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy b
Abstract
Hemangioendothelioma (HE) is a rare vascular tumor of endothelial cell origin that usually involves long bone or soft tissue. However, there are cases of head and neck involvement. In the literature, only 19 cases of nasal involvement are described.We report a case of an 18-month-old child who had HE of the ethmoid sinus. Endoscopic resection of the tumor was performed.The patient is well without any recurrence after 4 years of follow-up.Because of its rarity, the literature on HE in the nasal cavity and paranasal sinus is reviewed and its management is discussed. D 2006 Elsevier Inc. All rights reserved.
1. Introduction
2. Case report
The term hemangioendothelioma (HE) was introduced by Mallory [1] in 1908. It became a useful empirical designation for vascular tumors of endothelial origin, histologically intermediate in appearance between hemangiomas and angiosarcomas and biologically intermediate in behavior between benign and fully malignant tumors [2,3]. Hemangioendothelioma is a rare tumor that usually involves long bones or soft tissue of the extremities [4]. However, there also exist cases of head and neck involvement. Cole [5] described 75 head and neck cases from 1965 to 1982. Involvement of the nose and paranasal sinus is very rare. In this article, we present a case of nasal HE in a 18-month-old child treated by endoscopic resection. Because of its rarity, the literature of HE in the nasal cavity and paranasal sinus is reviewed, and the management is discussed.
An 18-month-old child, AN, affected by bilateral nasal obstruction, recurrent epistaxis, and sleep apnea, was admitted to another department. A mass was observed in the right nasal fossa. A biopsy did not furnish significant results and led to epistaxis causing severe anemia, necessitating several blood transfusions. The patient was transferred to our Division, and endoscopic nasal examination revealed a wine-colored neoformation occupying the whole of the right nasal fossa. Thin-section (2 mm) CT scan with contrast medium, demonstrated an enhancing expansile mass in the right nasal cavity. It also revealed a scalloping of the medial wall of the maxillary sinus, and that of the orbit was also evident. Magnetic resonance imaging (MRI) demonstrated the presence of a fairly homogeneous signal intensity mass, hypointense on T1-weighted images and hyperintense on T2-weighted images, with flow-void images inside. Hypertrophy of the homolateral sphenopalatine artery was also evident (Fig. 1). Angiography showed a hypervascularized lesion with a capillary bed supplied by the right spheno-palatine artery. A presurgery selective embolization with polyvynilic alcohol particles was then performed (Fig. 2); the following day, the patient underwent endoscopic resection of the mass. Cottonoids
4 Corresponding author. Clinica Otorinolaringoiatrica, IRCCS Policlinico S. Matteo, P. le Golgi 2, 27100 Pavia, Italy. Tel.: +39 3479484550; fax: +39 0382 528184. E-mail address: [email protected] (L. Semino). 0196-0709/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.11.006
288
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
Fig. 3. Microscopic examination (hematoxylin eosin 10) shows a tumor characterized by elongated, arborizing blood vessels lined by monomorphic, strikingly protuberant (hobnail) endothelial cells, with limited cytoplasm and rare vacuolation. Mitotic figures are not present. All of these features are suggestive of an HE. Fig. 1. Coronal T1-weighted MRI scan with contrast, preoperative study showing a contrast-enhancing mass occupying the entire right nasal cavity, with flow-void areas inside.
soaked in oxymetazoline solution were positioned in the nasal cavity and left in place for 10 minutes. One percent lidocaine with 1:200 000 epinephrine was subsequently injected at the level of the root of the middle turbinate and of the uncinate process. The specimen was resected ben bloc,Q with care taken that the dissection was carried out in the subperiosteal plane. The middle turbinate was removed too. Anterior and posterior ethmoidectomy, a large middle antrostomy, and sphenoidotomy were performed. There was little intraoperative bleeding, and the neoplasm was removed transorally. The intraoperatory biopsy excluded the presence of a malignant neoplasia. The ethmoid sinus was packed with absorbable hemostatic gelatine sponge and the nasal cavity, with polyurethane foam dressing. The nasal packing was removed on the second day. The postoperative recovery period was uneventful. Blood transfusions were unnecessary. The patient
Fig. 2. Angiography in anteroposterior plane. A, A hypervascularized tumor with an unhomogenous blush and the presence of an arteriocapillary circulation supplied by the right sphenopalatine artery. B, A postembolization angiogram shows a complete devascularization of the tumor.
was discharged 3 days later. Microscopic examination showed a tumor characterized by elongated, arborizing blood vessels lined by monomorphic, strikingly protuberant (hobnail) endothelial cells, with limited cytoplasm and rare vacuolation. Mitotic figures were not present. All of these features were suggestive of an HE (Fig. 3). Immunohistochemical staining for endothelial-related antigens (Factor VIII, CD31, and CD34) confirmed the diagnosis of HE. All resection margins of the tumor were found to be negative for neoplastic involvement. Considering the histological results and the age of the patient, it was decided not to submit the patient to postoperative radiation therapy.
Fig. 4. Coronal T1-weighted MRI scan with contrast, postoperative study showing no recurrence of original tumor.
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
289
Table 1 Case reports of sinonasal HE (a review of English literature) Site
Sex
Age
Treatment
Follow-up
Author
M M
3 mo 13
Surgery Radiotherapy
F F M M F
22 29 56 71 47
Radiotherapy Radiotherapy, Surgery Radiotherapy Radiotherapy Radiotherapy Surgery
M M F M M M F M M F
8 9 29 63 38 63 26 41 10 24
18 19
Maxillary sinus Middle meatus
M M
25 23
Surgery Surgery 2 Surgery Radiotherapy, surgery Surgery 2 Radiotherapy Radiotherapy Chemiotherapy, radiotherapy Surgery Surgery 3, Cryosurgery 7, Radiotherapy, Chemiotherapy Surgery, Radiotherapy Surgery
Alive, no recurrence at 3 y No response to treatment, lost to follow-up Alive, no recurrence at 3 y Died at 4 y Alive with disease at 2 y Alive no recurrence at 5 y Died at 3 mo, cervical and lung metastasis Alive, no recurrence at 3 y Alive, no recurrence at 12 y Alive, no recurrence at 7 y Alive, no recurrence at 14 mo Alive, no recurrence at 8 y Alive, no recurrence at 40 mo Alive, no recurrence at 6 mo Alive, no recurrence at 10 mo Lost to FU Died at 1 y with recurrence and a second tumor Alive, no recurrence at 6 mo Alive, no recurrence at 1 y
Henny [7] McCarthy and Pack [8]
8 9 10 11 12 13 14 15 16 17
Maxillary sinus Maxillary sinus, ethmoidal, orbit Nares Maxillary sinus Ethmoids Posterior nasal cavity Maxillary sinus, ethmoid, orbit Nasal septum Nasal septum Nasal vestibule Maxillary sinus Nasal cavity nares Nares Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus
1 2 3 4 5 6 7
The patient was followed-up with endoscopic examination and MRI of the facial skeleton every 6 months (Fig. 4). Four years after surgery, there was no endoscopic or radiological sign of disease recurrence. 3. Discussion The biologic behavior of HE has been variously described over the years as a neoplasm with a low grade of malignancy with possibly benign behavior or as a neoplasm with highgrade malignancy [5]. Other authors have distinguished between a benign and a malignant form [6]. More recently, the term HE has been used to designate some vascular tumors of an endothelial origin that occupy an intermediate position between benign hemangioma and full-blown angiosarcoma [2,3]. This confusing classification scenario clearly contributes to the difficulty inherent in providing adequate evaluation of the treatment and prognosis of these lesions. Hemangioendothelioma may involve bone or soft tissues. Hemangioendothelioma within the head and neck is uncommon; but this area represents the second commonest site of origin after the extremities. The skin and soft tissues of the face, scalp, and neck are particularly prone to develop this tumor. Cole [5], in a review of literature on head and neck HE, described 75 cases. The most frequent site was the soft tissues of the scalp. The sinonasal localization is rare. From a review of English publications from 1974 to 2003, only 19 cases were reported (Table 1). Only 5 of these were children, their age ranging from 3 months to 13 years. The most frequently affected site was the maxillary sinus (10 cases). The clinical symptoms of nasal HE are no different from those of any other nasal tumor, and the diagnosis can only be made by a
McCarthy and Pack [8] McCarthy and Pack [8] McCarthy and Pack [8] McCarthy and Pack [8] Bomer and Arnold [9] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Karmody and Kim [10] McClatchey et al [11] Williams and Thomas [12] Sharma and Nawalkha [13] Yasuoka et al [14] Dass and Saleem [4] Di Girolamo et al [15]
histopathologic examination. Because of their peculiar histological features, they can often be misinterpreted as carcinomas or chondrosarcomas. However, their endothelial nature has been substantiated by electron microscopy and immunohistochemistry [4]. Wide excision appears to be the treatment of choice, whenever possible. Radiotherapy is quite effective in palliation as the tumor is still radiosensitive [5,16]. Chemotherapy has no effect on this type of tumor [17]. Therapeutic results for HE of paranasal sinus are more encouraging than in other sites [3]. The mortality rate for children seems to be better that of adults [18]. Various authors have proposed the use of the fiberoptic endoscope in the treatment of benign or low-grade malignant neoplasm of the sinonasal region [19-21]. In our case, the endoscopic technique permitted to remove the tumor bloc, following a semicircular dissection plane, starting the incision in healthy tissue, and always remaining in the subperiosteal plane. Moreover, the embolization allowed surgical removal with minimum bleeding, with no alteration of the tumoral cell ultrastructure and, therefore, of the histological specimen. The endoscopic treatment allowed the young patient to avoid suffering a demolitive and disfiguring surgical approach as well as external scars on the facial skin. We did not perform postoperative radiotherapy: according to Heliashar [16], irradiation is not an innocent procedure for a child. If the margins of the specimen are tumor-free, irradiation may be reserved for later stages if needed. In expert hands, the endoscopic approach offers a valid alternative to the traditional techniques in the removal of benign or low-grade malignant neoplasm. Therefore, we limited our approach to the minimally invasive endoscopic intervention to preserve the future skeletal development of
290
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
the maxilla of the young patient. Four years on, it can be stated that events justify this choice. Because of the confusing classification, along with the notoriously unpredictable biologic behavior of HE, close follow-up of the patient is undoubtedly required.
References [1] Mallory FB. The results of application of special histological methods to the study of the tumors. J Exp Med 1908;10:575 - 93. [2] Enzinger FM, Weiss SW. Hemangioendothelioma: vascular tumors of intermediate malignancy. In: Enzinger FM, Weiss SW, editors. Soft Tissue Tumors. 4th ed. St-Luis7 Mosby; 2001. p. 891 - 915. [3] Barnes L. Tumors and tumorlike lesions of soft tissues. In: Barnes L, editor. Surgical pathology of the head and neck. 2nd ed. New York7 Marcel Dekker; 2001. p. 984 - 6. [4] Dass AA, Saleem Y. Hemangioendothelioma of the maxillary sinus. Otolaryngol Head Neck Surg 1995;112:735 - 7. [5] Cole IE. Hemangioendothelioma of the head and neck. J Laryngol Otol 1982;96:545 - 58. [6] Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: a clinicopathologic study. I. General features and vascular tumors. Cancer 1974;33:1275 - 88. [7] Henny FA. Angiosarcoma of maxilla in 3 month old infant: report of a case. J Oral Surg 1949;7:250 - 2. [8] McCarthy WD, Pack GT. Malignant blood vessel tumors. Report of 56 cases of angiosarcoma and Kaposi’s sarcoma. Surg Gynecol Obstet 1950;91:465 - 82. [9] Bomer DL, Arnold GE. Rare tumors of the ear, nose and throat. Third series: uncommon malignant tumors of the head and neck. Acta Otolaryngol Suppl (Stockh) 1971;289:1 - 25.
[10] Karmody CS, Kim CH. Angiosarcoma of the premaxilla. Laryngoscope 1974;84:560 - 4. [11] McClatchey KD, Batsakis JG, Rice DH, et al. Angiosarcoma of the maxillary sinus: report of a case. J Oral Surg 1976;34:1019 - 21. [12] Williams RM, Thomas JN. An ultrastructural study of a nasal haemangioendothelioma. J Clin Pathol 1979;32:475 - 81. [13] Sharma BG, Nawalkha PL. Angiosarcoma of the maxillary antrum: report of a case with brief review of literature. J Laryngol Otol 1979; 93:181 - 6. [14] Yasuoka T, Okumura Y, Okuda T, et al. Hemangioma and Malignant Hemangioendothelioma of the maxillary sinus. J Oral Maxillofac Surg 1990;48:877 - 81. [15] Di Girolamo A, Giacomini PG, Coli A, et al. Epitelioid haemangioendothelioma arising in the nasal cavity. J Laryngol Otol 2003; 117:75 - 7. [16] Heliashar R, Saah D, Osin P, et al. Hemangioendothelioma of the temporal bone in a child. Int J Pediatr Otorhinolaryngol 1997;40: 67 - 71. [17] Burgoon Jr CF, Soderberg M. Angiosarcoma. Arch Dermatol 1969;99:773 - 4. [18] Staut AP, Lattes R. Angiosarcomatoses. Tumor of the soft tissues. Atlas of tumor pathology (2th series). Washington (DC)7 Armed Forces Institute of Pathology; 1966 [fascicle 2]. [19] London SD, Schlosser RJ, Gross CW. Endoscopic management of benign sinonasal tumors: a decade of experience. Am J Rhinol 2002; 16:221 - 7. [20] Nicolai P, Berlucchi M, Tomenzoli D, et al. Endoscopic surgery for juvenile angiofibroma (JA). When and how. Laryngoscope 2003;119: 775 - 82. [21] Tomenzoli D, Castelnuovo P, Pagella F, et al. Different endoscopic surgical strategies in the management of inverted papilloma of the sinonasal tract: experience with 47 patients. Laryngoscope 2004;114: 193 - 200.
Endoscopic treatment of ethmoidal hemangioendothelioma: case report and review of the literature Lucia Semino, MDa,4, Fabio Pagella, MDa, Giovanni Delu`, MDb, Alessandra Todeschini, MDc, Ombretta Luinetti, MDd, Federico Zappoli, MDe, Paolo Castelnuovo, MDb a ENT Department, University of Pavia, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy ENT Department, University of Insubria, Ospedale di Circolo, Fondazione Macchi, 21100 Varese, Italy c Neuroradiology Department, Istituto Neurologico IRCCS Fondazione C. Mondino, 27100 Pavia, Italy d Department of Human Pathology, University of Pavia, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy e Department of Radiology, IRCCS Policlinico S. Matteo, 27100 Pavia, Italy b
Abstract
Hemangioendothelioma (HE) is a rare vascular tumor of endothelial cell origin that usually involves long bone or soft tissue. However, there are cases of head and neck involvement. In the literature, only 19 cases of nasal involvement are described.We report a case of an 18-month-old child who had HE of the ethmoid sinus. Endoscopic resection of the tumor was performed.The patient is well without any recurrence after 4 years of follow-up.Because of its rarity, the literature on HE in the nasal cavity and paranasal sinus is reviewed and its management is discussed. D 2006 Elsevier Inc. All rights reserved.
1. Introduction
2. Case report
The term hemangioendothelioma (HE) was introduced by Mallory [1] in 1908. It became a useful empirical designation for vascular tumors of endothelial origin, histologically intermediate in appearance between hemangiomas and angiosarcomas and biologically intermediate in behavior between benign and fully malignant tumors [2,3]. Hemangioendothelioma is a rare tumor that usually involves long bones or soft tissue of the extremities [4]. However, there also exist cases of head and neck involvement. Cole [5] described 75 head and neck cases from 1965 to 1982. Involvement of the nose and paranasal sinus is very rare. In this article, we present a case of nasal HE in a 18-month-old child treated by endoscopic resection. Because of its rarity, the literature of HE in the nasal cavity and paranasal sinus is reviewed, and the management is discussed.
An 18-month-old child, AN, affected by bilateral nasal obstruction, recurrent epistaxis, and sleep apnea, was admitted to another department. A mass was observed in the right nasal fossa. A biopsy did not furnish significant results and led to epistaxis causing severe anemia, necessitating several blood transfusions. The patient was transferred to our Division, and endoscopic nasal examination revealed a wine-colored neoformation occupying the whole of the right nasal fossa. Thin-section (2 mm) CT scan with contrast medium, demonstrated an enhancing expansile mass in the right nasal cavity. It also revealed a scalloping of the medial wall of the maxillary sinus, and that of the orbit was also evident. Magnetic resonance imaging (MRI) demonstrated the presence of a fairly homogeneous signal intensity mass, hypointense on T1-weighted images and hyperintense on T2-weighted images, with flow-void images inside. Hypertrophy of the homolateral sphenopalatine artery was also evident (Fig. 1). Angiography showed a hypervascularized lesion with a capillary bed supplied by the right spheno-palatine artery. A presurgery selective embolization with polyvynilic alcohol particles was then performed (Fig. 2); the following day, the patient underwent endoscopic resection of the mass. Cottonoids
4 Corresponding author. Clinica Otorinolaringoiatrica, IRCCS Policlinico S. Matteo, P. le Golgi 2, 27100 Pavia, Italy. Tel.: +39 3479484550; fax: +39 0382 528184. E-mail address: [email protected] (L. Semino). 0196-0709/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.11.006
288
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
Fig. 3. Microscopic examination (hematoxylin eosin 10) shows a tumor characterized by elongated, arborizing blood vessels lined by monomorphic, strikingly protuberant (hobnail) endothelial cells, with limited cytoplasm and rare vacuolation. Mitotic figures are not present. All of these features are suggestive of an HE. Fig. 1. Coronal T1-weighted MRI scan with contrast, preoperative study showing a contrast-enhancing mass occupying the entire right nasal cavity, with flow-void areas inside.
soaked in oxymetazoline solution were positioned in the nasal cavity and left in place for 10 minutes. One percent lidocaine with 1:200 000 epinephrine was subsequently injected at the level of the root of the middle turbinate and of the uncinate process. The specimen was resected ben bloc,Q with care taken that the dissection was carried out in the subperiosteal plane. The middle turbinate was removed too. Anterior and posterior ethmoidectomy, a large middle antrostomy, and sphenoidotomy were performed. There was little intraoperative bleeding, and the neoplasm was removed transorally. The intraoperatory biopsy excluded the presence of a malignant neoplasia. The ethmoid sinus was packed with absorbable hemostatic gelatine sponge and the nasal cavity, with polyurethane foam dressing. The nasal packing was removed on the second day. The postoperative recovery period was uneventful. Blood transfusions were unnecessary. The patient
Fig. 2. Angiography in anteroposterior plane. A, A hypervascularized tumor with an unhomogenous blush and the presence of an arteriocapillary circulation supplied by the right sphenopalatine artery. B, A postembolization angiogram shows a complete devascularization of the tumor.
was discharged 3 days later. Microscopic examination showed a tumor characterized by elongated, arborizing blood vessels lined by monomorphic, strikingly protuberant (hobnail) endothelial cells, with limited cytoplasm and rare vacuolation. Mitotic figures were not present. All of these features were suggestive of an HE (Fig. 3). Immunohistochemical staining for endothelial-related antigens (Factor VIII, CD31, and CD34) confirmed the diagnosis of HE. All resection margins of the tumor were found to be negative for neoplastic involvement. Considering the histological results and the age of the patient, it was decided not to submit the patient to postoperative radiation therapy.
Fig. 4. Coronal T1-weighted MRI scan with contrast, postoperative study showing no recurrence of original tumor.
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
289
Table 1 Case reports of sinonasal HE (a review of English literature) Site
Sex
Age
Treatment
Follow-up
Author
M M
3 mo 13
Surgery Radiotherapy
F F M M F
22 29 56 71 47
Radiotherapy Radiotherapy, Surgery Radiotherapy Radiotherapy Radiotherapy Surgery
M M F M M M F M M F
8 9 29 63 38 63 26 41 10 24
18 19
Maxillary sinus Middle meatus
M M
25 23
Surgery Surgery 2 Surgery Radiotherapy, surgery Surgery 2 Radiotherapy Radiotherapy Chemiotherapy, radiotherapy Surgery Surgery 3, Cryosurgery 7, Radiotherapy, Chemiotherapy Surgery, Radiotherapy Surgery
Alive, no recurrence at 3 y No response to treatment, lost to follow-up Alive, no recurrence at 3 y Died at 4 y Alive with disease at 2 y Alive no recurrence at 5 y Died at 3 mo, cervical and lung metastasis Alive, no recurrence at 3 y Alive, no recurrence at 12 y Alive, no recurrence at 7 y Alive, no recurrence at 14 mo Alive, no recurrence at 8 y Alive, no recurrence at 40 mo Alive, no recurrence at 6 mo Alive, no recurrence at 10 mo Lost to FU Died at 1 y with recurrence and a second tumor Alive, no recurrence at 6 mo Alive, no recurrence at 1 y
Henny [7] McCarthy and Pack [8]
8 9 10 11 12 13 14 15 16 17
Maxillary sinus Maxillary sinus, ethmoidal, orbit Nares Maxillary sinus Ethmoids Posterior nasal cavity Maxillary sinus, ethmoid, orbit Nasal septum Nasal septum Nasal vestibule Maxillary sinus Nasal cavity nares Nares Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus
1 2 3 4 5 6 7
The patient was followed-up with endoscopic examination and MRI of the facial skeleton every 6 months (Fig. 4). Four years after surgery, there was no endoscopic or radiological sign of disease recurrence. 3. Discussion The biologic behavior of HE has been variously described over the years as a neoplasm with a low grade of malignancy with possibly benign behavior or as a neoplasm with highgrade malignancy [5]. Other authors have distinguished between a benign and a malignant form [6]. More recently, the term HE has been used to designate some vascular tumors of an endothelial origin that occupy an intermediate position between benign hemangioma and full-blown angiosarcoma [2,3]. This confusing classification scenario clearly contributes to the difficulty inherent in providing adequate evaluation of the treatment and prognosis of these lesions. Hemangioendothelioma may involve bone or soft tissues. Hemangioendothelioma within the head and neck is uncommon; but this area represents the second commonest site of origin after the extremities. The skin and soft tissues of the face, scalp, and neck are particularly prone to develop this tumor. Cole [5], in a review of literature on head and neck HE, described 75 cases. The most frequent site was the soft tissues of the scalp. The sinonasal localization is rare. From a review of English publications from 1974 to 2003, only 19 cases were reported (Table 1). Only 5 of these were children, their age ranging from 3 months to 13 years. The most frequently affected site was the maxillary sinus (10 cases). The clinical symptoms of nasal HE are no different from those of any other nasal tumor, and the diagnosis can only be made by a
McCarthy and Pack [8] McCarthy and Pack [8] McCarthy and Pack [8] McCarthy and Pack [8] Bomer and Arnold [9] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Fu and Perzin [6] Karmody and Kim [10] McClatchey et al [11] Williams and Thomas [12] Sharma and Nawalkha [13] Yasuoka et al [14] Dass and Saleem [4] Di Girolamo et al [15]
histopathologic examination. Because of their peculiar histological features, they can often be misinterpreted as carcinomas or chondrosarcomas. However, their endothelial nature has been substantiated by electron microscopy and immunohistochemistry [4]. Wide excision appears to be the treatment of choice, whenever possible. Radiotherapy is quite effective in palliation as the tumor is still radiosensitive [5,16]. Chemotherapy has no effect on this type of tumor [17]. Therapeutic results for HE of paranasal sinus are more encouraging than in other sites [3]. The mortality rate for children seems to be better that of adults [18]. Various authors have proposed the use of the fiberoptic endoscope in the treatment of benign or low-grade malignant neoplasm of the sinonasal region [19-21]. In our case, the endoscopic technique permitted to remove the tumor bloc, following a semicircular dissection plane, starting the incision in healthy tissue, and always remaining in the subperiosteal plane. Moreover, the embolization allowed surgical removal with minimum bleeding, with no alteration of the tumoral cell ultrastructure and, therefore, of the histological specimen. The endoscopic treatment allowed the young patient to avoid suffering a demolitive and disfiguring surgical approach as well as external scars on the facial skin. We did not perform postoperative radiotherapy: according to Heliashar [16], irradiation is not an innocent procedure for a child. If the margins of the specimen are tumor-free, irradiation may be reserved for later stages if needed. In expert hands, the endoscopic approach offers a valid alternative to the traditional techniques in the removal of benign or low-grade malignant neoplasm. Therefore, we limited our approach to the minimally invasive endoscopic intervention to preserve the future skeletal development of
290
L. Semino et al. / American Journal of Otolaryngology–Head and Neck Medicine and Surgery 27 (2006) 287–290
the maxilla of the young patient. Four years on, it can be stated that events justify this choice. Because of the confusing classification, along with the notoriously unpredictable biologic behavior of HE, close follow-up of the patient is undoubtedly required.
References [1] Mallory FB. The results of application of special histological methods to the study of the tumors. J Exp Med 1908;10:575 - 93. [2] Enzinger FM, Weiss SW. Hemangioendothelioma: vascular tumors of intermediate malignancy. In: Enzinger FM, Weiss SW, editors. Soft Tissue Tumors. 4th ed. St-Luis7 Mosby; 2001. p. 891 - 915. [3] Barnes L. Tumors and tumorlike lesions of soft tissues. In: Barnes L, editor. Surgical pathology of the head and neck. 2nd ed. New York7 Marcel Dekker; 2001. p. 984 - 6. [4] Dass AA, Saleem Y. Hemangioendothelioma of the maxillary sinus. Otolaryngol Head Neck Surg 1995;112:735 - 7. [5] Cole IE. Hemangioendothelioma of the head and neck. J Laryngol Otol 1982;96:545 - 58. [6] Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: a clinicopathologic study. I. General features and vascular tumors. Cancer 1974;33:1275 - 88. [7] Henny FA. Angiosarcoma of maxilla in 3 month old infant: report of a case. J Oral Surg 1949;7:250 - 2. [8] McCarthy WD, Pack GT. Malignant blood vessel tumors. Report of 56 cases of angiosarcoma and Kaposi’s sarcoma. Surg Gynecol Obstet 1950;91:465 - 82. [9] Bomer DL, Arnold GE. Rare tumors of the ear, nose and throat. Third series: uncommon malignant tumors of the head and neck. Acta Otolaryngol Suppl (Stockh) 1971;289:1 - 25.
[10] Karmody CS, Kim CH. Angiosarcoma of the premaxilla. Laryngoscope 1974;84:560 - 4. [11] McClatchey KD, Batsakis JG, Rice DH, et al. Angiosarcoma of the maxillary sinus: report of a case. J Oral Surg 1976;34:1019 - 21. [12] Williams RM, Thomas JN. An ultrastructural study of a nasal haemangioendothelioma. J Clin Pathol 1979;32:475 - 81. [13] Sharma BG, Nawalkha PL. Angiosarcoma of the maxillary antrum: report of a case with brief review of literature. J Laryngol Otol 1979; 93:181 - 6. [14] Yasuoka T, Okumura Y, Okuda T, et al. Hemangioma and Malignant Hemangioendothelioma of the maxillary sinus. J Oral Maxillofac Surg 1990;48:877 - 81. [15] Di Girolamo A, Giacomini PG, Coli A, et al. Epitelioid haemangioendothelioma arising in the nasal cavity. J Laryngol Otol 2003; 117:75 - 7. [16] Heliashar R, Saah D, Osin P, et al. Hemangioendothelioma of the temporal bone in a child. Int J Pediatr Otorhinolaryngol 1997;40: 67 - 71. [17] Burgoon Jr CF, Soderberg M. Angiosarcoma. Arch Dermatol 1969;99:773 - 4. [18] Staut AP, Lattes R. Angiosarcomatoses. Tumor of the soft tissues. Atlas of tumor pathology (2th series). Washington (DC)7 Armed Forces Institute of Pathology; 1966 [fascicle 2]. [19] London SD, Schlosser RJ, Gross CW. Endoscopic management of benign sinonasal tumors: a decade of experience. Am J Rhinol 2002; 16:221 - 7. [20] Nicolai P, Berlucchi M, Tomenzoli D, et al. Endoscopic surgery for juvenile angiofibroma (JA). When and how. Laryngoscope 2003;119: 775 - 82. [21] Tomenzoli D, Castelnuovo P, Pagella F, et al. Different endoscopic surgical strategies in the management of inverted papilloma of the sinonasal tract: experience with 47 patients. Laryngoscope 2004;114: 193 - 200.