Mandibular ameloblastoma with intracerebral and pulmonary metastasis William V. Harrer, Philadelphia, Pa. POST
M.D.,* AND UNIVERSITY
and. Art&r SURGICAL
meloblastomas reportedly represent 1 per cent of all tumors of the jaw.ll z While multiple local recurrences occur in 50 to 72 per cent of reported cases,?, :i only seven cases of metastatic ameloblastoma have been reported. The following case report concerns a patient who had multiple recurrences with local excisions of a mandibular ameloblastoma, underwent a left hemimandibulectomy 15 years prior to death, and developed intracranial extension of the tumor and pulmonaq metastases. CASE REPORT Patient A. M., a 52-year-old Caucasian woman, was admitted to Thomas Jefferson University Hospital in November, 1968, because of symptoms secondary to metastatic amelo. blastoma. In 1915, when the patient was 29 years of age, a diagnosis of left mandibululnr ameloblastoma arising in a clentigerous cyst was made. During the next several years, t,here were multiple local recurrences and local excisions of this lesion. In 1953, 15 years prior to her final admission, the patient underwent a hemimandibulectomy at another institution. She was first admitted to Thomas Jefferson University Hospital in June, 1967, because oL vertigo, deafness, incoordination, and cough. A chest roentgenogram showed multipIt* pulmonary nodules. A skull film showed evidence of previous surgical intervention but no other abnormalities. Other x-ray studies yielded normal findings. In January, 1968, the patient was readmitted because of nausea, dyspnea, and left neck and facial pain. At that time, there was no evidence of local recurrence. Pulmonary nodules were still evident radiographically. A roentgenogram of the skull revealed destruction of the left sphenoi4 sinus. A chest film suggested an increase in the number of nodular pulmonary lesions. The patient’s final admission was in November, 1968, because of left facial pain, inability to swallow, and frequent cough. She appeared acutely ill and cachectic. She was cyanotic and dyspneic at rest. There was ptosis and severe conjunctivitis of the left eye. *Associate “*Assistant
in Pathology. Professor.
Fig. 1. Chest roentgenogram, final hospital ameloblastoma. met ;astases from mandibular
A left seventh nerve paralysis was present. There were no palpable cervical lymph nod A chest film showed partial collapse of the right middle lobe and an increase in size the pulmonary parenchymal nodules (Fig. 1). Therapy was begun with a combination imidazole and fluorouracil. The patient’s condition continued to deteriorate and pnem nitis developed. She died in December, 1968, 23 years after the initial diagnosis ameloblastoma. A complete postmortem examination was performed 12 hours after death. Pertint autopsy findings included recurrent tumor in the soft tissue of the left neck and base
Fig. 3. Ameloblastoma Magnification, x100.)
les. of of noof slit of
Fig. 5. Soft-tissue recurrence (Hematoxylin and eosin stain.
Oral Burg. June, 1970
in neck. Ameloblastoma Magnification, x400.)
the skull, infiltrating into the cranial cavity (Fig. 2), and metastatic ameloblastoma involving both lungs. The tumor involved the left sphenoid area and left cerebropontine angle, with compression of the fifth, sixth, seventh, eighth, ninth, and tenth cranial nerves on the left (Fig. 3). In addition to tumor metastasis, the lungs showed severe, acute bilateral bronchopneumonia. Histologically, this tumor had the classic pattern of ameloblastoma consisting of islands and strands of an odontogenic type of epithelium within a mature fibrous stroma (Fig. 4). Very few mitotic figures were seen. There were many areas of squamous metaplasia. Areas of central necrosis with microscopic cystic degeneration were noted in the local tumor recurrence and in the metastatic sites. The tumor was seen in small blood vessels and lymphatics (Fig. 5).
DISCUSSION Ameloblastomas are thought to arise from the epithelial lining of dentigerous cysts (as in the present case), remnants of dental lamina, enamel organ, or the basal layer of the oral mucous membrane.4 These tumors are locally invasive and can reach large size without evidence of metastasis.5 Seven cases of metastatic ameloblastoma have been previously reported+l’ and are summarized in Table I. In all but one of these casesthere were multiple local recurrences before there was evidence of metastasis. The interval between histologic diagnosis and proved metastasis has been from 3 to 34 years. It is of interest that seven of the eight reported cases have developed in the left jaw. No explanation for this is apparent. The predominant site of metastasis is the lung (five cases). Tumor aspiration has been suggested as the probable metastatic route.7j 8~I2 Cases of lymphatic6 and blood vessel extension*’ 8slo have also been reported. The present case demonstrates vascular invasion, although possible pulmonary tumor aspiration cannot be excluded. Direct extension of the tumor into the cranial cavity has been
Table I. Metastatic
Site of metastasis
Duration until death (wars i More than “3 (El)”
Four recurrences, hemimandil~alpc~ton~\
Ttventy-four local escisions, hemimnndibulectomy, radiun,
Seven Ioc,aI excisions, hemimandibulectom~, radiation therapy
Multiple recurrence:,, x-ray therapy. radium
More than 38 (a, b)”
Seven local recurrences, hemimandibulnctomy
Node at carotid bifurcation (Case 2)
Lung, maxilla, base of skull
Lee, White, and Tottens
Lung, pleura, orbit, brain
Tsukada, De La Pava, and Pickrenlo
Hoke and Harrelsonll
Lash and McCoy=
Marc than 3 (2 ) .;-
One local exeiaion, h~mimandihnl~cton~~
Harrer and Patchefsky
Multiple loc:~I clxc+ sions, hrtmimandit)ulcctomy, ctrc~motherapy
surgical procedures, x-ray therally
reportedsp s and was seen in this case. Histologically, tumors which will m&stasize cannot be differentiated from nonmetastasizing ameloblastomas.“, *‘I Ameloblastomas have a high recurrence rate. Local excision with a wide margin free of tumor is the treatment of choice. This may require hemimandibulectomy. Most patients with metastases have had multiple recurrences prior to the development of metastatic disease. Therefore, after incomplete remoua,l, as evidenced by locally recurrent tumor, earlier ablative surgery is intlic~atetl to prevent metastasis. SUMMARY A case in which a 52-year-old woman with a 23-year history of amcloblastoma was unsuccessfully treated by multiple local surgical excisions, a hemimandibulectomy 15 years prior to death, and finally chemotherapy is reported. This patient had widespread soft-tissue recurrence in the left neck, intracranial extension, and multiple parenchymal metastases. Evidence of vascular invasion was present. This represents the eighth reported ease of metastatic ameloblastoma and the third case of combined pulmonary and intracerebral extension. The secretarial R. I,ang,
authors acknowledge assistance of Miss M.D.
the photographic Barbara MacTough.
assistance of The postmortem
Ronald Picard and thr: was performed hy JV;1rreu
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