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ANGIOSARCOMA OF THE MAXILLARY SINUS: LITERATURE REVIEW AND CASE REPORT Murat Bankaci, MD, Eugene N. Myers, MD, Leon Barnes, MD, and Philip DuBois, MD

rare case of angiosarcoma of the maxillary sinus is reported. Review of the literature concerning angiosarcoma of the head and neck revealed 14 previously published cases involving the nose, nasal sinuses, and nasopharynx. Angiosarcoma of the skin and soft tissues of the head and neck differs in behavior from that affecting the nose, nasal sinuses,and nasopharynx. The case reported presents the protracted clinical course of this malignant tumor.

Abstract: A

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Malignant nonepithelial tumors of the nasal cavity, nasal sinuses, and nasopharynx are uncommon, comprising only 10% of malignant tumors occurring in this region. 18,19,29*37Although angiosarcoma has been regarded as a very rare neoplasm, a review of the literature from 1934 to 1949 revealed its incidence to be higher than had previously been ~ u p p o s e d .S~t~o*~~t~~clearly l , ~ ~established angiosarcoma as a malignancy of endothelial cells and set guidelines for differentiating it from a variety of other sarcomas. The tumor is characterized by the following histologic features: (1) the presence of irregular, freely communicating vascular channels; (2) lining of vascular spaces by endothelial cells with large, plump, hyperchromatic nuclei; and (3) foci of intravascular papillary projections of endothelial cells producing partial luminal obstruction. At times, the level of differentiation of tumor cells may be quite poor; indeed, subclassification of the tumor by origin from blood vessels, lymphatic vessels, or both has been complicated by

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Sinus Angiosarcoma

this problem, resulting in overlapping terminol0gy.17 Hence, it would be difficult to compile a list of published cases, since a number of different terms have frequently been used to define identical or similar pathologic entities.= Only 14 cases of angiosarcoma involving the maxillary sinus, nose, and nasopharynx have been The purpose of previously reported.z~9~11~17~1s~z1~30~34 this paper is to document an additional case involving the maxillary sinus and to review the literature on angiosarcoma of the head and neck. CASE REPORT

A 68-year-old white male was admitted to the Eye and Ear Hospital, Pittsburgh, in July 1976 with a history of right hemorrhagic rhinorrhea, facial pain, and diplopia. He had developed right nasal obstruction in November 1973 but did not seek medical attention until April 1974, when he developed foul hemorrhagic rhinorrhea, facial pain, and tenderness of the right midface. Physical examination demonstrated mucopurulent discharge from the right nasal cavity and “granulation tissue” beneath the inferior turbinate. Poor dental hygiene was also apparent. On radiologic examination of the nasal sinuses, opacity of the maxillary antrum and depression of the floor of the right orbit were reported. After one week of antibiotic treatment, a nasoantral irrigation was attempted. Destruction of the medial wall of the maxillary antrum was found a t that time. The possibility of a neoplasm was then raised. The

From the Department of Otolaryngology (Drs Bankaci and Myers), the Department of Pathology (Dr Barnes) and the Department of Radiology (Dr DuBois), University of Pittsburgh School of Medicine Eye and Ear Hospital Pittsburgh PA Address reprint requests to Dr Myers at the Department of Otolaryngol ogy, Eye and Ear Hospital, 230 Lothrop Street, Pittsburgh PA 15213 Accepted for publication May 8 1978 0148-64031010310274 $00 OOiO 1979 Houghton Mifflin Professional Publishers

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right maxillary sinus was therefore explored via a Caldwell-Luc approach. During the procedure, the surgeon noted destruction of the anterior and medial walls of the maxillary sinus. Purulent debris filled the antrum, and two teeth projected into the sinus. These teeth, a polypoid mass, and all of the lining of the maxillary antrum were removed. The specimen was submitted for histologic examination, which revealed “chronic suppurative inflammation and fibrosis.” The patient reported that the hemorrhagic rhinorrhea had persisted following surgical treatment, but that he had not returned to his physician for follow-up after the first postoperative visit. He had worked in a coal mine for 41 years, and a few years ago had discontinued his long-standing, heavy cigarette-smoking habit and had started chewing tobacco. He admitted to occasional alcohol intake. Physical examination showed a well-developed, wellnourished white male. Examination of the eyes showed right enophthalmos. It also revealed marked lower-lid induration (which brought up the question of destruction of the floor of the orbit) as well as a soft-tissue mass and paralysis of the right inferior rectus muscle. Nasal examination demonstrated hemorrhagic, purulent, foul-smelling discharge in the right nasal cavity and absence of the right lateral nasal wall. The right medial and inferior turbinates could not be identified. The nasal septum and left nasal cavity were normal. Oral examination showed a healed surgical scar in the right gingivobuccal sulcus. Palpation of the roof of the oral cavity revealed absence of the bony palate on the side, with only soft tissue covering the area. The nasopharynx could not be inspected. There was no organomegaly or lymphadenopathy. Expirative wheezes were heard over both lungs. RESULTS Laboratory Data. The following laboratory results were obtained: hemoglobin 10 g; hematocrit 33%; white blood cell count 5,800/mm3; prothrombin time (PT) 12 sec (control, 11 sec); partial thromboplastin time (F’TT) 76 sec (control, 29 sec); platelet count 366,000. Because of the increase in F’TT, the patient was evaluated for coagulopathy and was found to have a deficiency in factor XII. His factor XI1 was 1%’ N (normal is 50%-150% N). However, this abnormality was not felt to be the etiology of the hemorrhagic nasal discharge, because factor XI1 deficiency should not cause a bleeding disorder.

Plain-film and tomographic examination of the nasal sinuses was performed; pluridirectional tomography was used for the frontal and lateral projections. A soft-tissue density was demonstrated in the right maxillary antrum and nasal cavity, extending to the posRadiologic Examination.

Maxillary Sinus Angiosarcoma

terior choana. There was extensive bone destruction of the lateral wall, floor, and roof of the antrum, with partial destruction of the posterior and medial walls and hard palate (fig. 1). These findings were strongly suggestive of malignancy originating in the right maxillary antrum. Surgery. Multiple biopsies of the right maxillary sinus taken intranasally, however, were not diagnostic. Although we could not specifically document the malignant process in the maxilla, we decided that a maxillectomy should be performed to establish a diagnosis, to allow drainage from the lesion, to provide a means of observation, and, it was hoped, to cleanse the area of the foul, necrotic discharge. Lateral rhinotomy and right maxillectomy with preservation of the orbit and its contents were performed. Our preoperative evaluation, both radiographically and surgically, had indicated that, with the exception of a few spicules of bone, the palate was the only remaining area of the maxilla. Thus, the maxillectomy that we performed simply involved removal of a few shreds of remaining posterior wall as well as the palate. During the procedure, frozen-section examination of the periorbital tissue was also not diagnostic. The nasopharynx was free of tumor. Diagnosis was finally established by histologic examination of the surgical specimen.

The tumor was composed of freely anastomosing, small, capillarysized vascular channels lined by cells with pink cytoplasm and oval- to spindle-shaped hyperchromatic nuclei (fig. 2). Focally, there was piling up of the endothelial cells partially obstructing the lumen of vascular channels (fig. 3). Gomori’s reticulum stain demonstrated the proliferating cells to be located within the framework of the vessel, although in some areas neoplastic cells did penetrate the reticulum sheath (fig. 4). Infrequently, the tumor assumed a more solid appearance that obscured its vascular origin. Mitoses averaged 0-2 per high-power field. There was extensive invasion of the soft tissues of the cheek and facial bones (fig. 5). Considerable necrosis, a great deal of hemorrhage, and many blood clots were apparent. In two previous biopsies, only inflammatory debris and blood clots containing gram-positive rods and cocci had been identified, and there had been no evidence of a neoplasm. A review of the original tissue obtained by the prior Caldwell-Luc procedure did show a small focus of hemangiosarcoma.

Histopathologit Examination.

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Figure 1 . (A) Frontal tomogram. Note the soft-tissue mass in the right maxillary antrum and nght inferior meatus, with destrucbon of the nasoantral wall, the lateral antral wall, the alveolar process, and the orb/ta/ floor. (5) Frontal tomogram at a more posterior level. Note the rnucosal thickening in the sphenoid sinuses. The pterygoid processes are intact. A soft-tissue mass extends to the right posterior choana. (C) Lateral tomogram through the right maxillary antrum. Bony erosion of the posterior antral wall (arrowhead) indicates the probable malignant nature of the mass. Extensive destruction of the hard palate (arrows) is evident. Broken lines indicate the expected position of the orbital floor and the anterior antral wall, both of which were destroyed.

The patient was discharged to his home for convalescence. One month postoperatively, he returned with a hemorrhage from the right eye. There was a small dehiscence of the medial canthal area secondary to the surgery on this infected tissue; blood was coming from this area of dehiscence. Examination of the maxillary cavity showed persistence of the tumor in the area of the residual orbital contents, and biopsy and electrodesiccation of the tumor were performed under general anesthesia. The pathologic diagnosis at this time was consistent with angiosarcoma, but metastatic evaluation showed no evidence of dissemination. The patient underwent radiotherapy to the right maxilla, to the ethmoid and sphenoid sinuses, and to the right orbit, with shielding of the lens and anterior chambers. He received 4,500

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rads in 15 fractions given in three equal parts. A recent follow-up examination at 22 months revealed no evidence of local tumor recurrence or systemic spread. DISCUSSION

Angiosarcoma of the head and neck has been reported in skin, soft tissues, nose, nasal sinuses, nasopharynx, orbit, temporal bone, middle ear, larynx, and mandible. There are very few reported cases in the orbit, middle ear, temporal bone, larynx, and mandible; therefore, no conclusions can be drawn regarding the natural history and effects of various treatment modalities on the prognosis of this tumor at these sites.4.6.10.23,26,32,35,39,41,45,46 We will first review angiosarcoma of the nose, nasal sinuses, and naso-

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Figure 2 The tumor is composed of freely anastomosing vascular spaces lined by bizarre hyperchromaiic endoihelial cells Hematoxylin and eosin, x 125.

figure 3 . High-power view of the tumor exhibits intravascular papillary projections of neoplastic endothelial cells, producing partial luminal obstruction Hernatoxylin and eosin, x 400.

pharynx, and will then evaluate that of the skin and soft tissues. Angiosarcoma of the Nose, Nasal Sinuses, and Nasopharynx. Although bone is richly vascularized,

less than 1%of bone tumors are of vascular origin,6 and angiosarcoma of bone is very rare: in a review of patients treated a t the Hospital for Joint Diseases in New York between 1930 and 1970, only five cases were found. None of these tumors was located in the maxillary sinus.6 A review of the literature indicates 14 published cases of this neoplasm arising in the nasal cavity, nasal sinuses, and nasopharynx. Treatment modalities included surgery (two patients), radiation therapy (eight patients), or combined radiation and surgery (four patients). Three patients developed recurrent disease, but with further treatment all remained free of disease for periods of one to seven years. Of the total group of reported patients, five showed no evidence of disease, three were lost to follOw-up, One was alive with disease two Years later, and two died with the disease after at least one year.

Maxillary Sinus Angiosarcoma

figure 4 . Gomori’s reticulum stain shows tumor cells lying predominately within the reticulin sheaths of each vessel ( x 400).

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The etiology of angiosarcoma is not known. McCarthy and Packlo reported the onset of disease with pregnancy in two cases. They also stressed the possibility of transformation of benign angioma to angiosarcoma, especially following radiation therapy; others believe that this is only ~peculation.~ Increased incidences of angiosarcoma in Jewish and Italian families, as well as in patients with strong family histories of cancer, were mentioned in only one publication.3o The usual symptoms of angiosarcoma are nasal obstruction, headaches, facial pain, epistaxis, gradual facial swelling, and displacement of the eye. Radiologic examination should reveal a tumor mass. Bone destruction, if present, provides a clue as to the malignant nature of the lesion. Histopathologic diagnosis is difficult, especially in the presence of infection. Treatment must vary according to the location and stage of the disease. Similarly, results of treatment will vary depending on the lesion, the time interval between onset and definitive treatment, and the individual aggressiveness of a given tumor. l3 Wide surgical resection, when applicable, has been used successfully. Radiation therapy is found

Figure 5 Mahgnant endothehal cells have invaded bone, causing extensive osseous destruction Hernatoxylm and eosin, x 125

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to have a definite place in the curative treatment of angiosarcoma of this region; it may be used in conjunction with surgery and as an indispensable method of symptom palliation in advanced cases.3o Therapeutic results of treatment of angiosarcoma of the nose, nasal sinuses, and nasopharynx have been more encouraging than treatment of angiosarcoma of other regions. This factor is probably due to the low-grade nature of tumors in this region, and to a shorter interval between diagnosis and treatment.30 Angiosarcoma of Skin and Soft Tissues in the Head and Neck. These are rare malignancies; they ap-

pear to have a peculiar, unexplained predilection for elderly individuals. l3 The tumors present as bluish or violaceous plaques with indistinct margins, or as soft, painless, and compressible nodules. Bleeding is a late The etiology of these tumors is unknown, but radiation therapy and trauma have been implicated. One case of angiosarcoma occurred 18 months after the patient had received radiotherapy and carbon dioxide cryotherapy for a benign hemangioma; three cases of malignant vascular tumors following radiotherapy of a previously benign vascular tumor have been reported.'^^^ Trauma has been reported prior to the onset of angiosa~-coma,~ but ~ *in ~ ~no ~ ~instance ~ * ~ ~ has a causal relationship been proven.16 The resemblance of angiosarcoma to contusion most likely contributes to the assumption of a traumatic origin. Angiosarcoma of the skin may be well or poorly differentiated. Well-differentiated angiosarcoma occurs mainly in children and in young adults but has occasionally been seen in older individuals.* Tumors with essentially similar histology may develop in limbs affected with the chronic lymphedema that occasionally follows mastectomy. 40 Poorly differentiated angiosarcomas occur primarily in elderly individuals, and they are locally invasive and mutilating.20 We were able to find 89 reported cases of angiosarcoma involving skin and soft tissues of the head and neck.3,5,7,12-16,20,22,25.27,28,31,R3,36,-11.43,41,4i-49 Those cases reported by Girard et al,20Farr et a1,16 and land ell^^^ could not be presented as individual cases, because they were not reported separately. Of the other 51 cases, 47 are summarized in table 1. Treatment has been a challenge in these cases. Multiple failures are probably due to underestimation of this widely infiltrating sarcoma by surgeons and radiotherapists.16 Surgery

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Table 1. Cases of angiosarcoma of the head and neck, skin and soft tissue.’ Treatment and Results Treatmenta Surgery Radiation therapy Chemotherapy Combinations Surgeryiradiation therapy Surgeryichemotherapy Chemotherapyiradiation therapy Results Persistenceirecurrence No evidence of disease Living with disease Lost to follow-up Dead with disease Dead from other causes

No. of Patients

20 13 1

9 1 3 11 7 3 6 19 1

9 a t a taken from Bardwfl et Burgoon and Soderberg,5 Cardozo et a/,’ Caro and Stubenrauch,8Deutsch,’2Downing K i n m ~ n t . *Lane,28 ~ and Mallory ‘‘ Drucker,‘5Karmody and Miller and Moms,3’ Reed et Rosa! et Stout,4’ S u ~ r m o n d :Sweitzer ~ and Winer,*4Urbach and Feinerman,” Wetdman,48and Wilson-Jones 4g bOf 57 patients, 7 received no treatment and there were no treatment data available for 3 patients, hence the total of 47

is most commonly employed, especially in cases involving well-circumscribed lesions. Radical neck dissection is reserved for patients with metastasis to cervical lymph nodes in whom the primary le-

sion is under control or re~ectable.~ Radiotherapy has been a controversial treatment modality. Early reports commented that “radiation therapy was disappointing even as a means of palliat i ~ n . ” ~Recent ’ reports recommend 6,000- 7,000 rads applied to a field several centimeters larger than the gross limits of the tumor for lesions that are multicentric and for those having ill-defined margins.36 In 1974, a case of a premaxillary angiosarcoma treated with radiation therapy was reported;22three years later, follow-up revealed no evidence of disease. Chemotherapy has been found to be ineffe~tive.~ In contrast to angiosarcoma of the skin and soft tissues, angiosarcoma of the nose, nasal sinuses, and nasopharynx is characterized as occurring at a younger mean age; having a lower incidence of persistence, recurrence, and metastasis; having a higher salvage rate; showing a higher percentage of patients with no evidence of disease in follow-up; and having a lower mortality rate. The case report presented demonstrates the relatively “benign” clinical course of this malignant tumor in the nasal sinuses. The patient lived with the disease three years prior to any oncologic treatment. Following radical surgery, radiation therapy, and electrodesiccation, the patient has now been free of disease €or 22 months.

REFERENCES 1. Arena S: Somatic tissue tumors i n the head and neck. Trans Pa Acad Ophthalmol Otolaryngol 14:lOl-104, 1963. 2. Ash JE, Beck MR, Wilkes JD: Tumors of the upper respiratory tract and ear. In Atlas of Tumor Pathology, Section 4, FASC 12-13. Washington, DC, Armed Forces Institute of Pathology, 1964. 3. Bardwil JM, Mocega EE, Butler J J , e t al: Angiosarcomas of the head and neck region. A m J Surg 116:548-553, 1968. 4. Bundens WD Jr, Brighton C T Malignant hemangio-endothelioma of bone: report of 2 cases and review of the literature. J Bone Joint Surg (Am)74:762-772, 1965. 5. Burgoon CF Jr, Soderberg M: Angiosarcoma. Arch Dermatol 99:773-774, 1969. 6. Calhoun JJ: Malignant hemangio-endothelioma (angiosarcoma). Oral Surg 27:156-160, 1969. 7. Cardozo DW, Claud PL, Chen I, e t al: Cystic pulmonary metastasis-complicating angiosarcoma of the scalp. Calif Med 105210-214, 1966. 8. Car0 MR, Stubenrauch CH Jr: Hemangio-endothelioma of the skin. Arch Dermatol Syph 51:295-304, 1945. 9. Carter J H , Dickerson R, Needy C: Angiosarcoma of bone: a review of the literature and presentation of a case. A n n Surg 144:107-117, 1956. 10. Cerny L, Kraus J , Rozhold 0: Hemangiosarcoma of the middle ear. Cesk Otolaryngoi 21:254-256, 1972.

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11. Crowe SJ, Baylor J W Benign and malignant growths of the nasopharynx and their treatment with radium. Arch Surg 6:429-489, 1923. 12. Deutsch I: Hemangio-endothelioma: a case report. A J R 70:776-780, 1953. 13. Dorfman HD, Steiner GC, Jaffe H L Vascular tumors of the bone. H u m Pathol 2:349-376, 1971. 14. Downing JG, Mallory GK: Cavernous hemangioma and trauma. Arch Dermatol Syph 22:414-422, 1930. 15. Drucker V: Hemangio-endothelioma: a malignant tumor. Radiology 49:231-237, 1947. 16. Farr HW, Carandang CM, Huvos A G Malignant vascular tumors of the head and neck. A m J Surg 116:501-504, 1964. 17. Freilich EB, Coe GC:Angiosarcoma, case report and review of the literature. A m J Cancer 26269-275, 1936. 18. Fu YS, Perzin K H Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: a clinicopathologic study. I. General features and vascular tumors. Cancer 33:1275- 1288, 1974. 19. Geschickter CF: Tumors of the nasal and paranasal cavity. A m J Cancer 24:637-700, 1935. 20. Girard C, Johnson WC, Graham JH: Cutaneous angiosarcoma. Cancer 26:868-883, 1970. 21. Henny FA: Angiosarcoma of the maxilla in a 3-month-old infant: report of a case. J Oral Surg 7:250-252, 1949.

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22. Karmody CS, Kim C H Angiosarcoma of the premaxilla. Laryngoscope 84:560-564, 1974. 23. Kinkade JM Angiosarcoma of petrous portion of the temporal bone-report of a case. Ann Otol Rhinol Laryngol 57~235-240, 1948. 24. Kinkade JM: Angiosarcoma, a review of the literature. Ann Otol Rhinol Laryngol 58:159-167, 1949. 25. Kinmont PD: Angiosarcoma of the face. Proc R SOCMed 58:249-250, 1965. 26. Kuo T, Sayers CP, Rosai J Masson’s “vegetant intravascular hemangio-endothelioma”: a lesion often mistaken for angiosarcoma. Cancer 38:1227-1236, 1976. 27. Landells JW: Malignant tumors of the heart and blood vessels. In Raven RW (Editor): Cancer, Vol 2. London, Butterworth, 1958, pp 512-524. 28. Lane O G Cutaneous angiosarcoma with metastasis. Br J Cancer 6230-235, 1952. 29. Lewis JS: Sarcoma of the nasal cavity. Ann Otol Rhinol Laryngol 78:778-785, 1969. 30. McCarthy WD, Pack G T Malignant blood vessel tumors. Surg Gynecol Obstet 91:465-482, 1950. 31. Miller SH, Morris WJ: Angiosarcoma-report of an unusual case. Br J Plust Surg 27:182-185, 1974. 32. Mladick RA, Georgiade NG, Williams TG, e t al: Angiosarcoma of the mandible: case report. Plast Reconstr Surg 43~92-95,1969. 33. Pack G T Tumors of the blood and lymphatic vessels. In Pack GT, Ariel IM (Editors): Treatment of Cancer and Allied Diseases, 2nd ed. New York, Hoeber, 1960, p 115. 34. Pagano A: Angiosarcoma cavernoso del sen0 mascellare. Arch Ital Otol 41:310-323, 1930. 35. Rathmell TK, Sophocles A Laryngeal papilloma showing changes of Bowen’s disease and angiosarcoma. Arch Otolaryngol 90:352-355, 1969.

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36. Reed RJ,Palomeque FE, Hairston MA 111, e t al: Lymphangiosarcomas of the scalp. Arch Dermatol 94396-402, 1966. 37. Ringertz N: Pathology of malignant tumors arising in nasal and paranasal cavities and maxilla. Acta Otolaryngol (Suppl) 27:l-405, 1938. 38. Rosai J , Sumner HW, Kostianovsky M, e t al: Angiosarcoma of the skin, H u m Pathol 7:83-109, 1976. 39. Srivastava SP, Luthra UK, Srivastava VK, e t al: Angiosarcoma of the mandible (2 cases). Indian J Cancer 4254256, 1967. 40. Stewart FW, Treves N: Lymphangiosarcoma in postmastectomy lymphedema. Cancer 1:@-81, 1948. 41. Stout A P Hemangio-endothelioma: a tumor of blood vessels featuring vascular endothelial cells. Ann Surg 118: 445-464, 1943. 42. Stout A P Tumors of the blood and lymphatic vessels. In Pack GT, Ariel IM (Editors): Treatment of Cancer and Allied Diseases, 2nd ed. New York, Hoeber, 1960, p 130. 43. Suurmond D Hemangio-endothelioma (angioplastic sarcoma). Br J Dermatol 70:132-138, 1958. 44. Sweitzer SE, Winer LH: Hemangio-endothelioma. Arch Dermatol Syph 34:997- 1007, 1936. 45. Thomas KH, Doldman H M Oral Pathology, 5th ed. St Louis, C V Mosby, 1960, p 1399. 46. Toto PD, Lavieri J: Primary hemangiosarcomas of the jaw. Oral Surg 12:1459-1463, 1959. 47. Urbach F, Feinerman L: Angiosarcoma. Arch Dermatol 99774-776, 1969. 48. Weidman A1 Hemangio-endothelioma of skin with metastasis to liver, lung and lymph nodes. Arch Dermatol Syph 62:655-660, 1950. 49. Wilson-Jones E: Malignant hemangio-endothelioma of the skin. Br J Dermatol 76:21-39, 1964.

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Angiosarcoma of the maxillary sinus: literature review and case report.

SELECTED TOPICS ANGIOSARCOMA OF THE MAXILLARY SINUS: LITERATURE REVIEW AND CASE REPORT Murat Bankaci, MD, Eugene N. Myers, MD, Leon Barnes, MD, and P...
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