Oral angiosarcoma misdiagnosed as a pyogenic granuloma

J Oral

Moxillofoc

Surg

.56:488-491.

1998

Oral

Jo&R.

Angiosarcoma as a Pyogenic Mario Mufioz, Alonso del Hoyo,

Misdiagnosed Granuloma

MD, * Florencio Monje, PbD, f PbD,f and Rafael Martz’n-Granizo,

The physical examination showed a well-developed, wellnourished, obese man in no acute stress. He was afebrile and had a blood pressure of 145/80mm Hg and a pulse rate of 96 beats/mm Intraoral examination showed a nonulcerated, sessile, slightly painful, brown, 2 X 2-cm mass in the right retromolar trigone (Fig 1). No limitations of mouth opening or bleeding were noted. The remaining oral mucosa had a normal appearance. There was no cervicofacial lymphadenopathy. A clinical diagnosis of pyogenic granuloma was made. The patient was subsequently taken to the operating room, where, using local anesthesia, the mass was excised. Microscopically, the tissue was composed of vascular channels in a fibrous stroma, resembling granulation tissue (Fig 2). A histologic diagnosis of pyogenic granuloma was made by the pathologist. Fourteen days after the excision, the patient returned, complaining of recurrence. At that time, a reddish mass accompanied by bleeding on minor trauma was observed (Fig 3). A panoramic radiograph disclosed a small, ill-defined, osteolytic lesion in the posterior mandibular alveolar crest (Fig 4). A biopsy was performed under local anesthesia, and heavy bleeding was encountered. Microscopically, part of the tumor had a similar pattern to that of the previous biopsy, with prominent irregular and interconnecting vascular channels, but there were proliferating atypical cells between them. However, in most of the areas the pattern was solid, with proliferating, short, spindleshaped cells with nuclear atypia evenly grouped and dis persed among irregular vascular slits (Figs 5, 6). The

Soft tissues sarcomas are uncommon in the head and neck.l Angiosarcoma is one of the rarest forms of soft tissue sarcoma, constituting less than 1% of all sarcomas.z It is composed of endothelial cells of either lymphatic or blood vessel origin and presents dithcult diagnostic and therapeutic problems.3,4 The tumor has been described in locations such as the spleen, long bones, liver, mammary glands, striated muscle, skin, and subcutaneous tissue.5 Its occurrence as a primary or metastatic tumor in the oral cavity is extremely unusual. In fact, only 4% have been found in the pharynx, oral cavity, or paranasal sinuses.6,7 Other terms, such as hemangioendotheliosarcoma, malignant hemangioblastoma, telangiectatic sarcoma, and angiofibrosarcoma, have been used to describe this tumor.g10 In this report, we describe a tumor that was initially misdiagnosed histologically as a pyogenic granuloma. However, the subsequent clinical course and additional histologic examination resulted in a diagnosis of angiosarcoma. This case illustrates the difEculty of establishing a definitive microscopic and clinical diagnosis of oral angiosarcoma.

Report

MD,f

of Case

A 6%year-old white man presented in June 1991 with a mass on the right posterior edentulousmandibularridge. He had first noticed the mass 1 week previously, and it had enlarged since then. The patient was otherwise in good health. The medical history indicated that hc had had an epidermoid carcinoma of the left anterior floor of the mouth fl2NlMO) 13 years previously, that was treated by local excision and radical neck dissection with no recurrence.

*Resident, Department of Maxillofacial Hospital and Autonoma University of Madrid, tHead,

Department

of Maxillofacial

Surgery, La Princesa

Spain. Surgery, Tnfanta

Cristina

Hospital and Extremadura University of Badajoz, Spain. *Professor, Department of Surgery, Antonoma University, Madrid, Spain. §StaffSurgeon, Clinic0 Hospital of Madrid, Spain. Address correspondence General Ricardos, 171,2-B, 0 1998

American

Association

and reprint requests 28025 Madrid, Spain. of Oral and Maxillofacial

to Dr Mtioz:

c/

Surgeons

FIGURE retromolar

0278.2391,‘98,‘5604-0025$3.00,‘0

488

1. Preoperative trigone.

appearance

of the intraoral

tumor

in the right

M&O2

489

ET AI

FIGURE 2. A, Photomicrograph showing that the lesional pattern at low power mimicks granulation tissue as in a pyogenic granuloma (HematoxJineosin, original magnification x 10). 6, Photomicrograph showing an nonspecific, mixed inflammatory infiltrate between irregular vessels and extensive edema (Hematoxylin-eosin, original magnification X40).

immunophenotype of the neoplastic cells was endothelial. Most of the cells were positive for factor VIII and vimentin and negative for keratin. These data supported the vascular origin of the tumor. A diagnosis of angiosarcoma was made. Under general anesthesia, the patient underwent a partial mandibulectomy. A mandibular reconstruction bone plate was placed, and a local flap was used to close the intraoral

FIGURE 3. first recurrence.

Intraoral

view

of the

soft tissue defect. Microscopically, the tumor showed the same patterns found in the second biopsy specimen, and the diagnosis of angiosarcoma was confnmed. The patient was discharged from the hospital 7 days later with appointments for further evaluation. One month later, the patient developed a lesion in the tonsillar area and right floor of the mouth with similar characteristics to the previous lesion.

490

ANGIOSARCOMA

MIMICKING

AS A PYOGENIC

GRANULOMA

The lesion was considered inoperable. Chemotherapy or radiation therapy were refused by the patient. The lesion spread to pharynx and larynx, and the patient died in August 1991. Permission for a postmortem examination was denied.

Discussion

FIGURE radiolucency

4.

Panoramic radiograph showing a [arrow) of the right mandibular alveolar

small, process.

ill-defined

Angiosarcoma of the head and neck is rare; however, when it does occur it is most commonly found in the skin of the scalp and face of elderly patients.2 This phenomenon is in contrast to the deep location of most other soft tissue sarcomas7 Fifty-three cases have been reported in the oral region, usually involv-

FIGURE 5. Photomicrograph showing thot the tumor pattern is similar to the one shown in Figure 2A in some areas. However, amona ” the vascular channels. aroliferating cells with slight otypio can be recognized (Hematoxylineosin, original magnification x401.

FIGURE

6. Photomicrogroph showing the predominant pattern of the tumor. Note the atypical mitotic figures in this field. Atypicot, short, spindle-shaped cells ore evenly grouped among the irregular vosculor slits [Hemotoxylin-eosin, original magnification x60).

MUr;rOZ

491

ET AL

ing the mandible. l1 Angiosarcoma generally occurs in the fifth and sixth decades of life. In our case, the patient was 68 years old, one of the oldest cases reported with presentation in the oral cavity.12 The macroscopic and microscopic appearance of this tumor can lead to a misdiagnosis of pyogenic granuloma. The clinical differential diagnosis should include pyogenic granuloma, giant cell granuloma, Kaposi sarcoma, hemangioma, and malignant melanoma. Likewise, the microscopic differential diagnosis should include hemangioma, hemangiopericytoma, papillary endothelial hyperplasia, angiolymphoid hyperplasia with eosinophilia, Kaposi sarcoma, malignant melanoma, metastatic renal cell carcinoma, and pyogenic granuloma.3 A diagnosis of angiosarcoma cannot always be made on the basis of the clinical findings. The final diagnosis can only be made based on a combination of the clinical findings with the histologic and immunohistochemical features.9J0 Diagnostic scrutiny on the pathologist’s part is important even when the surgeon believes that a common lesion is being submitted for microscopic analysis. The current case, as well as those cases previously reported, indicate that angiosarcoma of the oral soft tissue is an aggressive neoplasm that results in frequent local recurrence, early metastasis, and a low survival rate.3 Two histologic findings that help to explain the frequent local recurrence of this tumor after surgical treatment are the poorly defined borders and early blood vessel proliferation far from the main tumor mass (multicentricity).2 The tumor tends to grow by widespread local invasion without welldefined gross boundaries. 13-16In fact, it seems possible that in this case the lesion began in the oral mucosa and spread to the mandible, but the reverse cannot be eliminated. Because so few cases of angiosarcoma of the oral cavity have been reported, there is a lack of long-term follow-up, and evaluation of the wide variety of

methods of therapy used is not possible. However, the current treatment of choice is wide excision.2,9J0 Neck dissection should be considered for patients with metastasis to the cervical lymph nodes.12 Radiation therapy and chemotherapy can be useful as palliative treatment.*‘J*

References 1. Freedman AM, R&man HM, Woods JE: Soft tissue sarcomas of the head and neck. Am J Surg 158:367, 1989 2. BardwiB JM, Mocega EE, Butler JJ: Angiosarcomas of the head and neck region. Am J Surg 116:548, 1968 JH, Fee WE: Angiosarcoma of the head and neck. 3. Cochran Otolaryngol Head Neck Surg 87:409, 1979 4. Frick WG, McDaniel K: Angiosarcoma of the tongue. J Oral Maxillofac Surg 46:496, 1988 5. Maddox JC, Evans HL: Angiosarcoma of skin and soft tissue: A study of forty-four cases. Cancer 48:1907, 1981 6. Toth BB, Fleming TJ, Lomba JA, et al: Angiosarcoma metastatic to the maxillary tuberosity gingiva. Oral Surg Oral Med Oral Path01 52:71, 1981 of disseminated angiosar7. Carr RJ, Green DM: Oral presentation coma. Br J Oral MaxiIlofac Surg 24:277,1986 8. Stout AP, Lattes R: Atlas of Tumor Pathology, series 2. Washington, DC, Armed Forces Institute of Pathology, 1966, pp 145-151 9. Enzinger FM, Weiss SW: Soft Tissue Tumors. St Louis, MO, Mosby, 1988, pp 545-561 10. Lucas RB: Pathology of Tumors of the Oral Tissues. New York, NY, ChurchiII Livingstone, 1976, pp 2 15-232 11. Sengun D, Tuncer I, Ertem N, et al: Metastatic hemangioendothehosarcoma of the mandible: Report of a case. J Oral Maxillofac Surg 44:806, 1986 12. Oliver AJ, Gibbons SD, Radden BG, et al: Primary angiosarcoma of the oral cavity. Br J Oral MaxiUofac Surg 29:38, 1991 JM: Angiosarcoma of petrous portion of temporal 13. Kinkade bone. Ann Otolatyngol57:235, 1948 14. Mladick RA, Georgiade NG, Williams TG: Angiosarcoma of mandible. Plast Reconstr Surg 43:92, 1969 15. Chow RW, Wilson CB, Olsen ER: Angiosarcoma of the skull. Cancer 25:902, 1970 16. Girard C, Johnson WC, Graham JM: Cutaneous angiosarcoma. Cancer 26:868,1970 CF, Sodeberg M: Angiosarcoma. Arch Dermatol 99: 17. Burgoon 773,1969 18. Zakrezewska JM: Angiosarcoma of the maxilla: A case report and review of the literature including angiosarcoma of maxillary sinus. Br J Oral MaxiUofac Surg 24:286, 1986