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The role of endoscopic sinus surgery in the diagnosis and treatment of metastatic orbital carcinoid tumors.

Abstract

Carcinoid tumor metastatic to the orbit is a rare occurrence. We report such a case in a patient with a carcinoid metastasis that was found in the medial rectus muscle. The diagnosis was established via a transnasal endoscopic approach. We also discuss the treatment of metastatic orbital carcinoid in the hope that we will increase awareness of the utility of transnasal endoscopy in facilitating both the diagnosis and treatment of posteromedial orbital pathology.

Introduction

Carcinoid tumors are slowly growing, potentially malignant neoplasms that arise from wherever endodermally derived neuroendocrine cells are found. The term carcinoid dates back to 1907, when Oberndorfer used it to describe a tumor whose cells exhibit a seemingly benign biologic behavior but are histologically similar to carcinoma cells. (1) It was not until several decades later that the malignant potential of these tumors became apparent.

Approximately 90% of carcinoid tumors originate in the gastrointestinal tract; other locations of particular interest to otolaryngologists include the middle ear, parotid gland, larynx, and thyroid gland. (2-5)

Orbital and ocular involvement by metastatic carcinoid is very rare. We describe a case in which the carcinoid tumor was metastatic to the medial rectus muscle. The diagnosis was made possible by endoscopic techniques. The purpose of this article is to increase awareness among otolaryngologists of the utility of the transnasal endoscopic approach in facilitating the diagnosis and treatment of posteromedial orbital pathology.

Case report

A 58-year-old woman presented to her ophthalmologist with a 6-week history of episodic vision loss in the fight eye and a 2-week history of increasing photophobia and right-sided retro-orbital pressure. Further inquiry revealed a 1-year history of fortification spectra across her visual field, and a 3-month history of paroxysmal vertex headaches radiating down the fight side of her head in the distribution of the ophthalmic and maxillary divisions of the ipsilateral trigeminal nerve. Her medical history was remarkable for metastatic carcinoid of the liver, which had been diagnosed 1 year earlier by liver biopsy. She had undergone cycles of cisplatin, mitomycin C, and doxorubicin intra-arterially to induce remission of her hepatic disease.

The patient was also experiencing episodic facial flushing and dyspnea suggestive of carcinoid syndrome, which provided a further impetus for a metastatic workup. The workup included a bone scan, echocardiogram, and an indium 111 ((111) In) octreotide scan. Radiolabeled octreotide is a somatostatin analog that is highly sensitive in detecting carcinoid tumors, and it identified an asymptomatic deposit in the lower lobe of the right lung, possibly indicating that this was the site of the primary tumor.

On ophthalmologic examination, the patient's visual acuity in the fight eye was 20/25, and the intraocular tension was normal. Restricted abduction was noted on the right, causing diplopia on lateral gaze. Fundoscopy revealed optic disk edema, but no afferent pupillary defect was seen. There was no ptosis or proptosis. Computed tomography (CT) of the orbit identified a 1.7 x 2.5-cm dumbbell-shaped mass in the right medial rectus muscle (figure 1). The mass extended to the orbital apex and displaced the optic nerve superiorly and laterally. Prominent scalloping of the lamina papyracea was visible, but there was no bony erosion or evidence of intracranial extension.

[FIGURE 1 OMITTED]

The patient's ophthalmic surgeon obtained a biopsy specimen via a right anterior orbitotomy. Unfortunately, not enough tissue was available to allow for a diagnosis, so the patient subsequently underwent a transnasal transethmoid endoscopic biopsy and limited orbital decompression (figure 2). Following a standard endoscopic middle maxillary antrostomy and total ethmoidectomy, the ipsilateral lamina papyracea was removed. The orbital periosteum was incised over the readily visible mass, and representative tumor samples were obtained.

[FIGURE 2 OMITTED]

Grossly, the tumor mass was pink-white, multilobular, soft, and friable. Infiltration of the medial rectus muscle fibers was readily apparent. Metastatic orbital carcinoid was diagnosed. Histologic examination revealed nests of small, cohesive cells with uniform nuclei, speckled chromatin, and indistinct or absent nucleoli typical of carcinoid tumors (figure 3). Immunostains demonstrated uniform cytoplasmic expression of synaptophysin and chromogranin, also characteristic of carcinoid tumors.

[FIGURE 3 OMITTED]

The patient's vision improved immediately after the procedure, only to progressively fade and become gradually darker over the following several weeks. Attempts to halt this progression with oral prednisone were unsuccessful, and repeat CT of the orbits revealed no change in the appearance of the mass. At 9 months' follow-up, the patient had completed 4,400 cGy of radiation to the right orbit, which produced a small decrease in the CT appearance of the tumor and mild clinical improvement in her sense of light perception. She was maintained on (111) In octreotide scanning to treat symptoms of associated carcinoid syndrome and cycles of palliative capecitabine chemotherapy. At 2-year follow-up, the patient was still alive with metastatic carcinoid.

Discussion

The orbit can host a great variety of primary and secondary neoplasms. The medial hemiorbit, defined as the area medial to the optic nerve, accounts for one-third of all orbital neoplasms. Within this area, a similarly large variety of possible pathologies can exist, but hemangioma, dermoid, and pleomorphic adenoma tend to occur in the lateral orbit.

A patient's age and sex can help develop a differential diagnosis in the case of a space-occupying lesion in the medial orbit. For example, children tend to be affected by rhabdomyosarcoma or optic nerve glioma, whereas the elderly are more likely to present with lymphoma or expansile sinus carcinoma. Women are more likely to present with meningioma or metastatic breast adenocarcinoma, whereas men are more likely to present with carcinoma.

Metastatic lesions account for 3 to 9% of all orbital tumors, with metastatic breast adenocarcinoma being the most common. (6) Carcinoid tumors metastasizing to the ocular structures or orbit are rare. When they do occur, they are nearly twice as common in women than men. (7)

All carcinoids are slowly growing, potentially malignant tumors. The most common site of origin is the appendix, followed by the ileum and bronchus. Being of neuroendocrine origin, carcinoid tumors can be nonfunctional or hormonally functional, and they can give rise to a variety of syndromes, including carcinoid syndrome, Zollinger-Ellison syndrome, Cushing's syndrome, and others. The classic features of carcinoid syndrome--episodic flushing, bronchospasm, diarrhea, and lower-extremity edema--are caused by serotonin release into the circulation. Fewer than 10% of patients with carcinoid tumor develop carcinoid syndrome; its presence usually indicates significant hepatic metastasis. (8) Death from carcinoid tumor can result from complications related to a metastasis or from the effects of serotonin on the right side of the heart.

Carcinoid tumors are histologically characterized by nests of cells with uniform small nuclei, indistinct nucleoli, and infrequent mitoses separated by delicate fibrous stroma. Cytoplasmic neurosecretory granules are highlighted by immunostains for synaptophysin and chromogranin. The pathologic differential diagnosis includes other metastatic neuroendocrine tumors (atypical carcinoid, pulmonary small-cell carcinoma, and Merkel cell carcinoma), meta-static melanoma, sinonasal small-cell undifferentiated carcinoma, olfactory neuroblastoma, and paraganglioma. Retinoblastomas and metastatic neuroblastomas, although histologically similar to carcinoid, primarily affect children.

Excluding metastases to the globe, only 26 cases of metastasis to the orbit have been previously reported in the literature (table). (7,9-23) Carcinoid metastases to the ocular structures or orbit usually spread from a primary in the midgut. (7) The less common bronchial carcinoids, which have a higher rate of metastasis, are more likely to spread to the uveal structures of the eye.

In view of the fact that so few cases have been reported, no consensus exists on the appropriate management of metastatic orbital carcinoid. Patients with orbital metastases have displayed a wide range of survival outcomes (table). This fact should be considered when choosing from among the therapeutic options for patients with metastatic carcinoid. Surgery is recommended for smaller orbital metastases, but the role of curative surgery for larger lesions is undefined. The results of radiotherapy are less encouraging than the results of surgery, although patient selection bias might have played a role in the poor survival rates associated with radiation. The role of chemotherapy is unproven. Somatostatin analogs such as octreotide are used for symptomatic relief of symptoms associated with carcinoid syndrome. Finally, enucleation or orbital exenteration is generally reserved for larger tumors that are unresponsive to other therapeutic modalities.

Traditional surgical approaches to the medial orbit are familiar to otolaryngologists, as this route is commonly used for drainage of subperiosteal abscesses, ethmoid artery ligations for epistaxis, and various forms of maxillectomy. Exposure of medial lesions near the orbital apex often requires detaching the medial rectus muscle from its scleral insertion or combining the medial approach with a lateral orbitotomy. The latter technique is associated with increased morbidity, and most external approaches require a concomitant dacryocystorhinostomy (DCR). Use of the transcaruncular approach to the medial orbit would preclude the need for a DCR, but this approach is more familiar to ophthalmic surgeons than to otolaryngologists. (24)

Transnasal endoscopy is another diagnostic and therapeutic option for the management of medial orbital lesions. In our patient, the location of the mass in the posteromedial orbit made access via an external orbitotomy difficult. The transnasal endoscopic approach is ideally suited for performing a biopsy of orbital lesions along the medial aspect of the orbit, as first reported in the ophthalmology literature by Wein et al. (9) This approach allows otolaryngologists to biopsy a lesion for diagnostic purposes and to possibly even remove a tumor to provide definitive treatment.

Our decision to initially perform a biopsy on the orbital tumor was based on the intraoperative diagnostic uncertainty posed by frozen-section pathologic diagnosis and the fact that patients with carcinoid tumors have a high incidence of second primary malignant neoplasms. (25,26) Complete endoscopic resection was an option, but not without sacrifice of the medial rectus muscle and a further potential threat to the patient's vision. More "favorable" lesions, such as those appearing to be encapsulated, would be given greater consideration for immediate endoscopic removal. Such a procedure in our patient was still an option after pathologic confirmation and proper patient counseling regarding vision risks. If a tumor is impinging on the optic nerve posteriorly, the endoscopic approach can be used for decompression purposes. Initially, our patient's vision was affected only minimally, so limited decompression was performed by removing the bony lamina.

Metastatic orbital carcinoid tumor represents only one of many different types of pathology in the medial orbit that the endoscopic surgeon potentially can diagnose or treat via a transnasal approach. As for carcinoid tumors, the limited worldwide experience with them precludes a general recommendation for complete resection in all cases.

To the best of our knowledge, ours is the first case reported in the otolaryngology literature of a carcinoid tumor metastatic to the orbit that demonstrates the utility of a transnasal endoscopic approach in establishing a diagnosis of orbital carcinoid. The endoscopic approach is familiar to otolaryngologists, and it provides us with relatively safe and direct access to the posteromedial orbit with minimal morbidity. It is our hope that otolaryngologists will keep this approach in mind for the diagnosis or treatment of posteromedial orbital pathology.

References

(1.) Oberndorfer S. Uber die "kleinen Dunndarmcarcinome." Verhandl Dtsch Path Gesellsch 1907;11:112-16.

(2.) Godwin JD II. Carcinoid tumors: An analysis of 2837 cases. Cancer 1975;36:560-9.

(3.) Murphy GF, Pilch BZ, Dickersin GR, et al. Carcinoid tumor of the middle ear. Am J Clin Pathol 1980;73:816-23.

(4.) Chan SW, Sizeland AM. Carcinoid tumour of the parotid gland. Med J Aust 2000; 173:276-7.

(5.) Markel SF, Magielski JE, Beals TE Carcinoid tumor of the larynx. Arch Otolaryngol 1980;106:777-8.

(6.) Henderson JW. Orbital Tumors. Philadelphia: W.B. Saunders; 1973:474.

(7.) Couch DA, O'Halloran HS, Hainsworth KM, et al. Carcinoid metastasis to extraocular muscles: Case reports and review of the literature. Orbit 2000;19:263-9.

(8.) Sabiston DC Jr., ed. Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 14th ed. Philadelphia: W.B. Saunders; 1991:871.

(9.) Wein FB, Perry JD, Miller NR, et al. Pulmonary carcinoid tumor presenting with simultaneous orbital and intracranial metastases: Value of transnasal endoscopic orbital biopsy and decompression. Orbit 1999;18:267-72.

(10.) Honrubia FM, Davis WH, Moore MK, Elliott JH. Carcinoid syndrome with bilateral orbital metastases. Am J Ophthalmol 1971;72:1118-21.

(11.) Font RL, Ferry AP. Carcinoma metastatic to the eye and orbit III. A clinicopathologic study of 28 cases metastatic to the orbit. Cancer 1976;38:1326-35.

(12.) Fishman ML, Rosenthal S. Optic nerve metastasis from amediastinal carcinoid tumour. Br J Ophthalmol 1976;60:583-8.

(13.) Gandini R, MariscottiC, Cunietti E. [Ileo-cecal malignant carcinoid tumor with orbital metastases as initial symptoms. Anatomo-clinical study]. Minerva Med 1979;70:919-24.

(14.) Harris AL, Montgomery A. Orbital carcinoid tumor [letter]. Am J Ophthalmol 1980;90:875-7.

(15.) Rush JA, Waller RR, Campbell RJ. Orbital carcinoid tumor metastatic from the colon. Am J Ophthalmol 1980;89:636-40.

(16.) Divine RD, Anderson RL, Ossoinig KC. Metastatic carcinoid unresponsive to radiation therapy presenting as a lacrimal fossa mass. Ophthalmology 1982;89:516-20.

(17.) Krohel GB, Perry S, Hepler RS. Acute hypertension with orbital carcinoid tumor. Arch Ophthalmol 1982;100:106-8.

(18.) Riddle PJ, Font RL, Zimmerman LE. Carcinoid tumors of the eye and orbit. A clinicopathologic study of 15 cases, with histochemical and electron microscopic observations. Hum Pathol 1982;13: 459-69.

(19.) Shields CL, Shields JA, Eagle RC, et al. Orbital metastasis from a carcinoid tumor: Computed tomography, magnetic resonance imaging, and electron microscopic findings. Arch Ophthalmol 1987; 105:968-71.

(20.) Shetlar DJ, Font RL, Ordonez N, et al. A clinicopathologic study of three carcinoid tumors metastatic to the orbit. Immunohistochemical, ultrastructural, and DNA flow cytometric studies. Ophthalmology 1990;97:257-64.

(21.) Fan JT, Buettner H, Bartley GB, Boiling JP. Clinical features and treatment of seven patients with carcinoid tumor metastatic to the eye and orbit. Am J Ophthalmol 1995;119:211-18.

(22.) Isidori AM, Kaltsas G, Frajese V, et al. Ocular metastases secondary to carcinoid tumors: The utility of imaging with [(123)I]meta-iodobenzylguanidine and [(111)In]DTPA pentetreotide. J Clin Endocrinol Metab 2002;87:1627-33.

(23.) Takemoto Y, Nishida N, Kojiro S, et al. Metastatic carcinoid tumor in the orbit. Kurume Med J 2003;50:165-7.

(24.) Shorr N, Baylis HI, Goldberg RA, Perry JD. Transcaruncular approach to the medial orbit and orbital apex. Ophthalmology 2000; 107:1459-63.

(25.) Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH. Life history of the carcinoid tumor of the small intestine. Cancer 1961;14: 901-12.

(26.)Kuiper DH, Gracie WA Jr., Pollard HM. Twenty years of gastro-intestinal carcinoids. Cancer 1970;25:1424-30.

Roland Z. Gerencer, MD; Uresh Patel, MD; Charles Hunter, MD; J. Timothy Heffernan, MD

From New Mexico Ear, Nose, and Throat Specialists, PC, Albuquerque (Dr. Gerencer); Seattle Radiologists, PC (Dr. Patel); Washington Pathology Consultants, PS, Seattle (Dr. Hunter); and Eye Associates Northwest, PC, Seattle (Dr. Heffernan).

Reprint requests: Roland Z. Gerencer, MD, New Mexico Ear, Nose, and Throat Specialists, PC, 401 Edith Blvd., N.E., Suite 210, Albuquerque, NM 87102. Phone: (505) 243-8030; fax: (505) 842-1158; e-mail: snot.doc@comcast.net
Table. Carcinoid tumors metastatic to the orbit: Summary of reported
cases

Author Specific location Treatment Outcome

Honrubia et al, Not specified Surgery A/W @ 2 yr
(10) 1971

Font and Ferry, Inferior rectus Enucleation Died of tumor
(11) 1976 muscle @ 11 yr

Fishman and Optic nerve Biopsy Died of tumor
Rosenthal, @ 4 wk
(12) 1976

Gandini et al, Bilateral Biopsy Died of tumor
(13) 1979 metastases @ 3 yr

Harris and Inferior rectus Radiotherapy Died of tumor
Montgomery, muscle @ 2 yr
(14) 1980

Rush et al, Superior and Radiotherapy A/W @ 12 mo
(15) 1980 inferior rectus
 muscles

Divine et al, Lacrimal fossa Radiotherapy Died of tumor
(16) 1982 @ 6 mo

Krohel et al, Bilateral Surgery and A/W @ 12 mo
(17) 1982 metastases chemotherapy

Riddle et al, Inferior rectus Enucleation Died of tumor
(18) 1982 muscle @ 9 yr
 Not specified Enucleation Died of CAD
 @ 4 yr
 Not specified Enucleation A/W @ 4 mo
 Not specified Surgery Died of tumor
 @ 4 yr
 Not specified Biopsy Died of tumor
 @ 2 yr
 Not specified Biopsy Lost to
 follow-up
Shields et al, Superior rectus Surgery A/W @ 7 mo
(19) 1987 muscle

Shetlar et al, Inferior rectus Surgery Died of tumor
(20) 1990 muscle @ 5 mo
 Not specified Surgery A/W @ 7.5 yr
 Not specified Surgery A/W @ 12 mo

Fan et al, Not specified Radiotherapy Died of tumor
(21) 1995 @ 6 mo
 Not specified Radiotherapy Died of tumor
 and @ 2 yr
 chemotherapy

 Superior rectus Chemotherapy Died of tumor
 muscle @ 2 yr

Wein et al, Medial and Surgery, A/W @ 4 mo
(9) 1999 inferior rectus radiotherapy,
 muscles and
 chemotherapy

Couch et al, Superior rectus Surgery A/W @ 2 yr
(7) 2000 muscle
 Medial rectus Surgery and Died of tumor
 muscle radiotherapy @ 14 yr

Isidori et al, Not specified Radiotherapy A/W @ 1.5 yr
(22) 2002

Takemoto et al, Superior orbit Surgery Lost to
(23) 2003 follow-up

Gerencer et al, Medial rectus Surgery, A/W @ 2 yr
* 2007 muscle radiotherapy,
 and
 chemotherapy

* Present case.

Key: A/W = alive and well: CAD = coronary artery disease.
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Author:Heffernan, J. Timothy
Publication:Ear, Nose and Throat Journal
Article Type:Disease/Disorder overview
Date:Mar 1, 2007
Words:2834
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