CASE REPORT Dennis H. Kraus, MD, Section Editor
A CASE OF CERVICAL METASTASES FROM TEMPORAL BONE CARCINOID Raul Pellini, MD,1 Marzia Ruggieri, MD,1 Barbara Pichi, MD,1 Renato Covello, MD,2 Giovanni Danesi, MD,3 Giuseppe Spriano, MD1 1 Department of Otolaryngology Head & Neck Surgery, National Cancer Institute ‘‘Regina Elena,’’ Via E. Chianesi 53, 00144 Rome, Italy. E-mail:
[email protected] 2 Department of Pathology, National Cancer Institute ‘‘Regina Elena,’’ Rome, Italy 3 Department of Otolaryngology, Desenzano del Garda Hospital, Desenzano del Garda, BS, Italy
Accepted 21 January 2005 Published online 11 April 2005 in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/hed.20197
Abstract: Background. Carcinoids are neuroendocrine tumors that typically occur in the gastrointestinal tract and lung and less frequently in the head and neck region. Whereas the metastatic potential of laryngeal carcinoid is well documented, only one case of metastasis of temporal bone carcinoid to the cervical lymph nodes has been reported. We present the case of a 55-year-old woman with cervical metastases almost 3 years after a primary diagnosis of temporal bone carcinoid. Methods. The patient had undergone a subtotal petrosectomy for a temporal bone carcinoid and, 29 months later, revision surgery for a local recurrence. Four months later, multiple laterocervical swelling appeared, and the patient underwent modified radical neck dissection. Results. Histologic examination after the neck dissection was suggestive of lymph node metastases from a carcinoid tumor. Immunohistochemical analysis of the lymph node metastases showed neoplastic cells positive for keratin, vimentin, chromogranin, and neuron-specific enolase and negative for thyroglobulin, calcitonin, S-100, and parathyroid hormone. The patient was treated with intensity-modulated radiation therapy and neck irradiation. Conclusions. We believe that temporal bone carcinoids have metastatic potential not predictable by histologic features.
Correspondence to: R. Pellini B 2005 Wiley Periodicals, Inc.
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Surgical excision is the treatment of choice for patients with temporal bone carcinoids, with the approach and technique determined by the extent of the mass. Moreover, before planning surgery and during follow-up, neck node status must be carefully detected. A 2005 Wiley Periodicals, Inc. Head Neck 27: 644 – 647, 2005 Keywords: atypical carcinoid; carcinoid; metastases; middle ear; temporal bone
Carcinoid tumors originate from neuroendocrine cells; frequent sites of occurrence are the gastrointestinal tract, lungs, pancreas, and ovaries. Ninety percent of these tumors originate from the enterochromaffin-Kulchitsky cells of the gastrointestinal tract. These cells are part of the system of amine precursor uptake and decarboxylation (APUD). They can synthesize, store, and secrete a variety of chemical messengers (serotonin and norepinephrine) and neuropeptides (adrenocorticotropic hormone, calcitonin, and antidiuretic hormone), which are responsible for the carcinoid syndrome. Head and neck neuroendocrine tumors are rare, and a correct evaluation of their real in-
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cidence is difficult. They have been previously described under a variety of names because of their different nature and biologic behavior. According to an accurate literature review for the period 1980 to 1999,1 only 37 cases of middle ear carcinoid tumors have been reported. Up until 1998, only 500 cases of laryngeal neuroendocrine tumors were reported (including epithelial tumors such as carcinoids and tumors of nervous origin like paraganglioma).2 Only 25 cases of paranasal sinus tumors were reported up until 2001.3 As with laryngeal carcinoids, various authors have described the locoregional aggressiveness of paranasal sinus carcinoids and the metastatic potential of these tumors through the lymphatic routes.2,4 To date, one only case of temporal bone carcinoid, described by Mooney et al,5 had a metastatic lymph node recurrence. It is evident that because of the limited number of cases of temporal bone carcinoid reported, it is not possible to either predict the biologic behavior of the tumor itself or to define a single therapeutic strategy. We present the clinical case of a patient with cervical carcinoid metastases that appeared nearly 3 years after the primary diagnosis of temporal bone carcinoid.
CASE REPORT
The patient, a 55-year-old woman, had a history since 1995 of imbalance, left-sided hearing loss, and tinnitus, followed by diplopia and blepharospasm. These symptoms worsened, and in October 2000, a CT scan showed a 3.5-cm, solid, enhanced lesion involving the petrous pyramid, with extensive erosion of the lateral wall of the external auditory canal. The mass involved the body and the big wing of the sphenoid up to the clivus. A petrosectomy was performed with an infratemporal fossa type C Fisch approach. The definitive histologic examination was suggestive of a carcinoid tumor. In March 2003, after a recurrence, the patient underwent a further operation to remove a gross mass occupying the infratemporal fossa and the parapharyngeal space. No laterocervical swellings were reported. The histologic examination confirmed the diagnosis of a carcinoid tumor recurrence. In July 2003, the patient was seen with multiple left cervical swellings. The clinical examination showed the outcomes of the previous subtotal petrosectomy, a depression of the left temporal-
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zygomatic area, and a VII cranial nerve paralysis (grade III, House – Brakman scale). A 3-cm, leftsided cervical mass, mobile on the superficial and deep layers, was localized at the II cervical level. Two more similar but smaller swellings were localized at level V. Head and neck CT scan and echotomography confirmed multiple pathologic lymph nodes at levels I, II, III, IV, and V on the left, the largest measuring 2.5 cm. There was no evidence of temporal bone recurrence on MRI. A fine-needle aspiration biopsy was performed. The cytologic pattern showed a population of cells with bland nuclear chromatin and inconspicuous nucleoli, suggestive of a neuroendocrine neoplasm. The patient did not show a carcinoid syndrome; gastrointestinal (diarrhea) and cardiovascular (valvular disease) symptoms and flushing were absent. The concentration of urinary 5-hydroxyindolacetic (serotonin hematic metabolite excreted by kidneys) was normal. Further examinations included chest CT scan, pelvic and renal ultrasonography, esophagogastroduodenoscopy (EGDS), and positron emission tomography (PET) scan to exclude another primitive carcinoid in another site. CT scan, ultrasonography, and EGDS did not reveal any pathologic findings, whereas the PET scan showed multiple sites of higher uptake on the left side of the neck. Thereafter, a modified radical neck dissection of levels I –V was performed. Recovery was uneventful, and the patient was discharged on the eighth postoperative day. The patient was treated with postoperative intensity modulated radiation therapy (IMRT) of the skull base, to a total dose of 70 Gy, of ipsilateral neck irradiation, to a total dose of 50 Gy (200 cGy per fraction). The definitive histologic examination showed metastases from a neuroendocrine tumor in 29 of 51 lymph nodes removed. Tumor cells were arranged in cords, nests, and glandular or rosette-like structures. The neoplastic cells had the classic features of carcinoid tumor, with small uniform nuclei having finely stippled chromatin. The cytoplasm varied from clear to eosinophilic. The tumor lacked cellular pleomorphism, necrosis, and mitotic figures (Figures 1 and 2). Immunohistochemical analysis of the lymph node metastases showed neoplastic cells that were positive for keratin, vimentin, chromogranin, and neuron-specific enolase and negative for thyroglobulin, calcitonin, S-100, and parathyroid hormone.
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FIGURE 1. Photomicrograph shows lymph node metastases by carcinoid tumor (neck dissection) (hematoxylin and eosin stain, original magnification 10). [Color figure can be viewed in the online issue, which is available at www.interscience.wiley.com.]
A review of the original biopsy specimens (taken in 2000) from the middle ear showed similar features. DISCUSSION
Middle ear carcinoid tumor is a rare lesion. It was described for the first time in 1980 by Murphy et al.6 In the head and neck region, the laryngeal localization seems to be more frequent than middle ear carcinoid, which has a lower metastatic potential but a higher incidence of local recurrence.7 Because of this different pattern of biologic behavior, middle ear carcinoid is considered more ‘‘benign.’’ In the case of neck nodal carcinoid metastases from middle ear carcinoid, it is necessary to exclude the existence of a second primary tumor in other sites such as the larynx, lung, gastrointestinal tract, and kidney.5 Torske and Thompson.8 retrospectively reviewed a series of 48 cases of benign middle ear neuroendocrine tumors (including carcinoid). In their study, no cases of lymph node metastases were reported. According to the authors, carcinoid tumor and middle ear adenoma represent the same tumor with different degrees of glandular and neuroendocrine differentiation. Moreover, the authors stated that, considering the low metastatic potential and local aggressiveness, these tumors should be regarded as benign. In their series, the tumors ranged from 0.2 to 3 cm in diameter. Moreover, lesions in most of the cases filled the tympanic cavity; in two cases, the tumors extended into the external auditory canal
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FIGURE 2. Photomicrograph shows extensive lymph node metastases by carcinoid tumor (neck dissection) (hematoxylin and eosin stain, original magnification 40). [Color figure can be viewed in the online issue, which is available at www.interscience. wiley.com.]
alone; in one case, into the external auditory canal and mastoid bone; in one case, into the mastoid bone alone; in one case, into the mastoid bone and eustachian tube; and in one case, into the eustachian tube alone. According to Torske and Thompson,8 the absence of metastases of middle ear tumors could be related to the small size of the neoplasm (average, 0.8 cm in their series) and to the relative lack of vascularity of the middle ear cavity compared with the lung. Still, according to Torske and Thompson,8 the only case of cervical metastases reported by Mooney et al5 could be regarded as a case of radiation-induced malignant transformation. In this case, in fact, the patient had been treated with partial resection of a glomus jugular tumor (probably a misdiagnosed carcinoid tumor) and external beam radiation therapy. Cervical metastases had appeared 8 years after the previous diagnosis of the middle ear tumor. Our patient was found to have recurrent disease in neck nodes 3 years after the diagnosis of the primary tumor. After a literature review, we can state that there are no other cases of petrous carcinoid cervical metastases except the one described by Mooney et al.5 In our case, we could explain the presence of neck metastases by the gross extension of the primary tumor and by the event of recurrence. The tumor showed a maximum diameter of 4.7 cm and from the petrous apex reached the media cranial fossa to the clivus, involving the parapharyngeal space and the pterygoid muscles.
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Surgical excision represents the treatment of choice for patients with a temporal bone carcinoid. The approach and the technique should be determined by the extension of the mass. We agree with Torske and Thompson8 that recurrences are due to an initial nonradical removal of the tumor. Considering the high potential of local recurrence, an initial complete removal of tumor with petrosectomy if necessary is always advisable. In case of a large tumor involving the infratemporal region, we believe that a prophylactic selective neck dissection of levels II –IV (mostly exposed by the surgical approach) should be recommended. In the case of a smaller tumor in which a subtotal petrosectomy is performed, a careful follow-up of the cervical lymph nodes is advisable. Postoperative irradiation at the time of the first excision was not considered because of the poor radiosensitivity of the tumor 5 and our effort to avoid the risk of developing a less differentiated and more aggressive radioinduced transformation. CONCLUSIONS
In conclusion, we believe that temporal bone carcinoid has a metastatic potential not predictable by the histologic features. Furthermore, we emphasize the importance of the role of electron microscopy and more so of immunohistochemical
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analysis to correctly diagnose and precisely evaluate the real incidence of the carcinoid tumor. Regional spread could be a late event related to tumor size, involvement of surrounding structures, and recurrence. In these selected cases, some type of surgical neck treatment and a careful follow-up should be recommended.
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