Case Report
AIII'is' Nasl/s' Larynx (Tokyo) 18, 307-314 (1991)
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA: A CASE REPORT Naoyuki KANOH, M.D., Yoshihiko NISHIMURA, M.D., Michiko NAKAMURA, M.D., Mutsuko MORI, M.D.,* and Kunio UEMATSU, M.D. * Department of Otolaryngology and *Department of Pathology (Hospital), Hyogo College of Medicine, Nishinomiya, Japan
We report a 49-year-old man with a paraganglioma involving the nasopharynx, which according to the literature is the fourth case in Japan and the twentieth in the world. The initial histopathologic findings of the biopsy specimen from the nasopharyngeal tumor suspected a carcinoid tumor. However, the final histopathologic diagnosis from the surgically resected specimen was paraganglioma, determined by electron microscopy and using several stains, such as Grimelius and Neuronspecific enolase (NSE) stains. We wish to add this case of "primary" nasopharyngeal paraganglioma to the literature, and to report the pathologic findings and clinical course of this unusual case. Paraganglionic tissue consists of collections of cells of neural crest OrIgm which are more extensively distributed in the fetus and newborn than in the adult. Tumors arising from paraganglionic tissue are far fewer than those originating in the major paraganglia of the head and neck. In some sites of paraganglioma, in particular in the nasal cavity and nasopharynx, the reporting of the presence of a paraganglioma preceded the identification of normal ganglia. Nasopharyngeal paragangliomas are extremely rare, with only 19 reported cases in the literature to our knowledge (SMITH, KOHUT, and MILLION, 1973; OTOKIDA, OHIRA, KAMIMURA, FUJIMORI, KATO, TAKAHASHI, SAIKI, KANAYA, TASHIRO, and ABE, 1987). The symptoms and signs of these reported cases included nasal obstruction and epistaxis (WEILLE and LANE, 1951; MORAN, 1962; PARISIER and SINCLAIR, 1968; HOUSE, GOODMAN, GACEK, and GREEN, 1972; SCOPPA and TONKIN, 1975), dysphonia (HOUSE et al., 1972), proptosis (SMITH et al., 1973; MORIMOTO, MATSUMOTO, YOSHIMINE, and MOGAMI, 1985), and otologic symptoms due to Eustachian tube blockage or direct invasion (WEILLE and LANE, 1951; DILL, 1959; Received for publication January 9,1991 307
308
N. KANOH
el
al.
SMITH et al., 1973). The symptoms and signs varied with tumor size and location. PEREZ, ACKERMAN, and MILL (1959) reported two cases of middle cranial fossa extension. All of the three Japanese cases presented at an advanced stage, with recognized middle cranial fossa invasion (MORIMOTO et al., 1985; OTOKIDA et al., 1987; OZAKURA, KIMURA, and KISHlMOTO, 1988). In contrast, in the present case, the tumor was detected at an early stage, and those clinical features were absent. CASE REPORT
A 49-year-old man consulted the Department of Otolaryngology, Hyogo College of Medicine, complaining of the sensation of a foreign body in the ph aryngolaryngeal region. At the first visit, the tumor occupying the posterior part of the vomer and anterior vault of nasopharyx was detected by ENT fiberscopy and posterior rhinoscopy; however, the patient showed no evidence of nasal blockage or dysphonia. The Eustachian tube orifices were intact, both eardrums appeared normal, and hearing was within normal limits. Histological examination of a biopsy specimen was suggestive of carcinoid tumor. Enhanced CT (Fig. 1) and MRI (Fig. 2) both indicated that the tumor also occupied the inferior part of the sphenoid sinus. There was no evidence of bony destruction of the base of the skull on CT scan or MRI. Resection of the entire tumor as well as the posterior part of the nasal septum and the surrounding normal mucosa was performed via a transpalatine approach, using Yeau's incision. Histopathologic examination of the resected specimen demonstrated paraganglioma. Three weeks
Fig. 1. Enhanced CT. (Sphenoid sinus is almost completely occupied by tumor.)
Fig. 2. MR image finding. (The arrows indicate the tumor shadow. There is no invasion of the base of the skull.)
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
309
after surgery the patient was noted to have good palatal movement and speech. Blood and urine tests including catecholamine and selotonine showed no significant abnormalities. Urinary 5-HIAA and VMA were also normal. During the clinical course, there were no episodes of flushing, hypertension, or diarrhea, which are characteristic of pheochromocytoma and carcinoid tumor. HISTOPATHOLOGY
The resected specimen was brown and measured 2.2 X 1.3 X 1.6 cm. It weighed 8 g.
Fig. 3. Clusters of round or oval clear cells adjacent to capillary blood vessels; "Zellballen" pattern. (H&E, original magnification x 100.)
Fig. 4. Dark-brown granules are observed in cytoplasm of tumor cells. (Grimelius stain, original magnification x 100.)
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Fig. 5. Tumor cells are strongly positive in cytoplasm. (NSE stain, original magnification x 150.)
Light microscopy of H&E stained sections of the lesion showed uniform ovoid clear cells separated by connective tissue together with vascular endothelial cells arranged in nests (Fig. 3) and the "Zellballen" pattern with an appearance similar to glomus tumor. Grimelius stain showed dark-brown secretory granules (Fig. 4) in the cytoplasm of the tumor cells. Neuron-specific enolase (NSE) stain was positive (Fig. 5), suggesting the presence of neuroendocrine cells. Other stains, including PAS, Chromogranin, and S-lOO protein, were all negative. The electron micrograph of glutalaldehyde-fixed tumor shown in Fig. 6 reveals the electron-dense granules (l00-150 nm). DISCUSSION
Paragangliomas ansmg in the head and neck are uncommon. The most common types are carotid body tumor, glomus tympanicum tumor, and glomus jugulare tumor (synonym: jugulotympanic paraganglioma). With respect to the site of origin of primary nasopharyngeal paraganglioma, GIRGIS and FAHMY (1973) speculated that paraganglionic tissue is normally present at birth surrounding the terminal part of the maxillary artery in the pterygopalatine fossa, and they suggested that the term "glomus nasopharyngis" should be applied to this group of tumors. Prior to this report, several nasopharyngeal paragangliomas arising in nearby paraganglia (e.g. jugulotympanic paraganglion, nodose paraganglion, and ciliary paraganglion) with possible extension into the nasopharynx were reported (SCOPPA and TONKIN, 1975; LACK, CUBILLA, WOODRUFF, and FARR, 1977; SCHULLER and LUCAS, 1982; CHAMBERS, NORMAN, DEDO, and FERRELL, 1982; KUHN and ARONOFF, 1989). Four cases of jugulotympanic paragangliomas were reported to
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
311
Fig. 6. Electron micrograph of the glutalaldehyde-fixed tumor demonstrating a small number of electron-dense granules (100-150 nm), suggesting the presence of neurosecretory granules.
312
N. KANOH et al.
exhibit the most aggressive local invasion (ASH and RAUM, 1949; WEILLE and LANE, 1951; DILL, 1959). Two nasopharyngeal paragangliomas originating from the nodose paraganglion origin have been reported by HOUSE et al. (1972). SMITH et al. (1973) described one case of ciliary ganglion origin. Taking into account the speculation of GIRGIS and FAHMY, as well as the location of the tumor, we suspect that the present tumor originated from the pterygopalatine ganglion, and thus can be classified as "glomus nasopharyngis." We propose that this kind of tumor should be termed "primary nasopharyngeal paraganglioma" to distinguish it from the nasopharyngeal extension of tumors arising from nearby structures. The differential diagnosis between carcinoid tumor and paraganglioma is difficult (BARNES and PEEL, 1990). Paragangliomas exhibit the pathological features of typical Zellballen pattern, whereas carcinoid tumors show a cordlike or trabecular pattern. In this case, an initial diagnosis of carcinoid tumor was made from the biopsy specimen. However, the final diagnosis of paraganglioma was made from examination of the surgically resected specimen, which showed typical cell nests and the Zellballen pattern using H&E and several other staining methods and electron micoscopy. Both carcinoid tumor and paraganglioma are considered to be functioning tumors; however in this case the clinical symptoms and signs and results of blood and urine tests were within normal limits. The previously reported cases of nasopharyngeal paragangliomas demonstrated no clinical functioning activity, with the exception of three recent cases (MATISHAK, SYMON, CHEESEMAN, and PAMPHLETT, 1987; OTOKlDA et al., 1987; OZAKURA et al., 1988). Whole-body MIBG (I-meta-iodobenzyl-guanidine) scintigraphy has been reported to be a useful method for detection of the primary lesion of catecholamine-secreting tumors (NAKAJO, SHAPIRO, Copp, KALFF, GROSS, SISSON, and BEIERWALTES, 1983). However, OZAKURA et al. (1988) reported that it was only successful in detecting the primary lesion in one case. The primary lesions in the other two cases were detected by performing selective venous blood collections and measuring for catecholamine concentrations. The only curative treatment for paraganglioma is adequate surgical excision of the tumor together with adjacent normal tissues. SCOPPA and TONKIN (1975) reported one case in which hemorrhage was marked and difficult to control during surgery. In order to achieve complete extirpation with detection of the feeding artery and minimization of blood loss, the three recent cases with large tumors underwent angiography and feeding artery embolization prior to surgery (OTOKIDA et al., 1987; MATISHAK et al., 1987; OZAKURA et al., 1988). New operative techniques, such as laser surgery and cryosurgery were also applied for tumor resection and control of bleeding. At least seven patients received irradiation therapy (ZAK and LAWSON, 1982). Chemotherapy was also administered in two cases (ZAK and LAWSON, 1982;
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
313
MORIMOTO et al. 1985). Morimoto achieved moderate results with chemotherapy, however, this was temporary. Fortunately, the tumor in our patient was detected at an early stage, and total resection could be performed without any complications. At present, neither postoperative irradiation nor chemotherapy is indicated, and careful follow-up examinations are regularly conducted. NSE stain in the present case was strongly positive. The NSE value of peripheral blood may be a good indicator of recurrence. CONCLUSION
We reported a rare case of nasopharyngeal paraganglioma with no clinical functioning activity. According to the literature, this was the fourth case in Japan, and the twentieth case in the world. To distinguish paraganglioma from nasopharyngeal extension arising from a nearby paraganglion, we term paragangliomas arising from the pterygopalatine ganglion "primary nasopharyngeal paraganglioma." We fortunately detected this tumor at an early stage, and were able to perform complete resection. This case emphasizes the importance of routine ENT examinations, especially in patients with abnormal findings in the nasopharynx. We are grateful for the kind advice of Prof. Takeo Kumoi and the English proofreading of Dr. Wendy Gray.
REFERENCES ASH, J. E., and RAUM, M.: An Atlas of Otolaryngic Pathology, 4th ed., Armed Forces Institute of Pathology, Washington, D.C., 1949. BARNES, L., and PEEL, R. L.: Head and Neck Pathology: A Text! Atlas of Differential Diagnosis, Chap 21: Paraganglioma versus carcinoid, pp. 45-46, Igaku-shoin, New York-Tokyo, 1990. CHAMBERS, E. F., NORMAN, D., DEDO, H. H., and FERRELL, L. D.: Primary nasopharyngeal chemodectoma. Neuroradiology 23: 285-288, 1982. DILL, J. L.: Tumors of the glomus jugular is. A report of nine cases. Laryngoscope 69: 248259, 1959. GIRGIS, 1. H., and FAHMY, S. A.: Nasopharyngeal fibroma; its histopathological nature. J. Ololaryngol. 0101. 87: 1107-1123, 1973. HOUSE, J. M., GOODMAN, M. L., GACEK, R. R., and GREEN, G. L.: Chemodectomas of the nasopharynx. Arch. Ololaryngol. 96: 138-141, 1972. KUHN, J. A., and ARONOFF, B. L.: Nasal and Nasopharyngeal Paraganglioma. J. Surg. Oncol. 40: 38-45, 1989. LACK, E. E., CUBILLA, A. L., WOODRUFF, J. M., and FARR, H. W.: Paragangliomas of the head and neck region. Cancer 39: 397-409, 1977. MATISHAK, M. Z., SYMON, L., CHEESEMAN, A., and PAMPHLETT, R.: Catecholamine-secreting paragangliomas of the base of the skull-Report of two cases. J. Neurosurg. 66: 604-608, 1987.
N. KANOH ef al.
314
MORAN, T. E.: Non-chromaffin paraganglioma of the nasal cavity. Laryngoscope 72: 201206,1962. MORIMOTO, K., MATSUMOTO, K ., YOSHIMINE, T., and MOGAMI, H.: Nasopharyngeal Paraganglioma extended to middle cranial fossa. Neurosurgery (in Japanese) 13, 805-809, 1985. NAKAJO, M., SHAPIRO, B., Copp, J., KALFF, V., GROSS, M. D., SISSON, J. C., and BEIERWALTES, W. H.: The norm~1 and abnormal distribution of the adrenomedullary imaging agent m-[I-1311 iodobenzylguanidine (1-131 MlBG) in man: Evaluation by scintigraphy. J. Nllc/. Med. 24: 672-682, 1983. OTOKIDA, K ., OHlRA, A., KAMIMURA, A., FUJIMORI, M ., KATO, M., TAKAHASHI, A ., SAIKI, I., KANAYA, H., TASHIRO, A., and ABE, T.: Cervical catecholamine- secreting paraganglioma in the pterygopalatine fossa. Tohokll J. Exp. Med. 153,347-354, 1987. OZAKURA, K., KIMURA, T., and KISHIMOTO, S. : Primary pterygopalatine catecholamine-secreting paraganglioma. H ead Neck Cancel' Jpn. (in Japanese) 15: 57, 1988. PARISIER, S. c., and SINCLAIR, G . M.: Glomus tumor of the nasal cavity. Laryngoscope 78: 2013-2024, 1968. PEREZ, C. A., ACKERMAN, L. V., and MILL, W. B.: Cancer of the nasopharynx: Factors influencing prognosis. Cancel' 24: 1-17, 1969. SCHULLER, D. E., and LUCAS, J. G . : Nasopharyngeal Paraganglioma- Report of a case and review of Iiterature- . Arch. 0 101. 108: 667-670, 1982. SCOPPA, J., and TONKIN, J . P.: Non-chromaffin paraganglioma of the nasopharynx. J . Laryngol. 89: 653-656, 1975. SMITH, C. J., KOHUT, R. r., and MILLION, R. R . : Chemodectomas of the nasopharynx. Laryngoscope 83: 330-335, 1973. WEILLE, F. L., and LANE, C. S.: Surgical problems involved in the removal of glomus-jugulare tumors. Laryngoscope 61, 448- 459, 1951. ZAK, F. G., and LAWSON, W. : Tumors of the Nose and Nasopharynx. The Paraganglionic Chemoreceptor System, pp. 276-285, Springer-Verlag, New York, 1982.
Request reprints to:
Dr. N. Kanoh, Department of Otolaryngology, Hyogo College of Medicine, I-I Mukogawa-cho, Nishinomiya 663, Japan
AIII'is' Nasl/s' Larynx (Tokyo) 18, 307-314 (1991)
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA: A CASE REPORT Naoyuki KANOH, M.D., Yoshihiko NISHIMURA, M.D., Michiko NAKAMURA, M.D., Mutsuko MORI, M.D.,* and Kunio UEMATSU, M.D. * Department of Otolaryngology and *Department of Pathology (Hospital), Hyogo College of Medicine, Nishinomiya, Japan
We report a 49-year-old man with a paraganglioma involving the nasopharynx, which according to the literature is the fourth case in Japan and the twentieth in the world. The initial histopathologic findings of the biopsy specimen from the nasopharyngeal tumor suspected a carcinoid tumor. However, the final histopathologic diagnosis from the surgically resected specimen was paraganglioma, determined by electron microscopy and using several stains, such as Grimelius and Neuronspecific enolase (NSE) stains. We wish to add this case of "primary" nasopharyngeal paraganglioma to the literature, and to report the pathologic findings and clinical course of this unusual case. Paraganglionic tissue consists of collections of cells of neural crest OrIgm which are more extensively distributed in the fetus and newborn than in the adult. Tumors arising from paraganglionic tissue are far fewer than those originating in the major paraganglia of the head and neck. In some sites of paraganglioma, in particular in the nasal cavity and nasopharynx, the reporting of the presence of a paraganglioma preceded the identification of normal ganglia. Nasopharyngeal paragangliomas are extremely rare, with only 19 reported cases in the literature to our knowledge (SMITH, KOHUT, and MILLION, 1973; OTOKIDA, OHIRA, KAMIMURA, FUJIMORI, KATO, TAKAHASHI, SAIKI, KANAYA, TASHIRO, and ABE, 1987). The symptoms and signs of these reported cases included nasal obstruction and epistaxis (WEILLE and LANE, 1951; MORAN, 1962; PARISIER and SINCLAIR, 1968; HOUSE, GOODMAN, GACEK, and GREEN, 1972; SCOPPA and TONKIN, 1975), dysphonia (HOUSE et al., 1972), proptosis (SMITH et al., 1973; MORIMOTO, MATSUMOTO, YOSHIMINE, and MOGAMI, 1985), and otologic symptoms due to Eustachian tube blockage or direct invasion (WEILLE and LANE, 1951; DILL, 1959; Received for publication January 9,1991 307
308
N. KANOH
el
al.
SMITH et al., 1973). The symptoms and signs varied with tumor size and location. PEREZ, ACKERMAN, and MILL (1959) reported two cases of middle cranial fossa extension. All of the three Japanese cases presented at an advanced stage, with recognized middle cranial fossa invasion (MORIMOTO et al., 1985; OTOKIDA et al., 1987; OZAKURA, KIMURA, and KISHlMOTO, 1988). In contrast, in the present case, the tumor was detected at an early stage, and those clinical features were absent. CASE REPORT
A 49-year-old man consulted the Department of Otolaryngology, Hyogo College of Medicine, complaining of the sensation of a foreign body in the ph aryngolaryngeal region. At the first visit, the tumor occupying the posterior part of the vomer and anterior vault of nasopharyx was detected by ENT fiberscopy and posterior rhinoscopy; however, the patient showed no evidence of nasal blockage or dysphonia. The Eustachian tube orifices were intact, both eardrums appeared normal, and hearing was within normal limits. Histological examination of a biopsy specimen was suggestive of carcinoid tumor. Enhanced CT (Fig. 1) and MRI (Fig. 2) both indicated that the tumor also occupied the inferior part of the sphenoid sinus. There was no evidence of bony destruction of the base of the skull on CT scan or MRI. Resection of the entire tumor as well as the posterior part of the nasal septum and the surrounding normal mucosa was performed via a transpalatine approach, using Yeau's incision. Histopathologic examination of the resected specimen demonstrated paraganglioma. Three weeks
Fig. 1. Enhanced CT. (Sphenoid sinus is almost completely occupied by tumor.)
Fig. 2. MR image finding. (The arrows indicate the tumor shadow. There is no invasion of the base of the skull.)
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
309
after surgery the patient was noted to have good palatal movement and speech. Blood and urine tests including catecholamine and selotonine showed no significant abnormalities. Urinary 5-HIAA and VMA were also normal. During the clinical course, there were no episodes of flushing, hypertension, or diarrhea, which are characteristic of pheochromocytoma and carcinoid tumor. HISTOPATHOLOGY
The resected specimen was brown and measured 2.2 X 1.3 X 1.6 cm. It weighed 8 g.
Fig. 3. Clusters of round or oval clear cells adjacent to capillary blood vessels; "Zellballen" pattern. (H&E, original magnification x 100.)
Fig. 4. Dark-brown granules are observed in cytoplasm of tumor cells. (Grimelius stain, original magnification x 100.)
310
N. KANOH et al.
Fig. 5. Tumor cells are strongly positive in cytoplasm. (NSE stain, original magnification x 150.)
Light microscopy of H&E stained sections of the lesion showed uniform ovoid clear cells separated by connective tissue together with vascular endothelial cells arranged in nests (Fig. 3) and the "Zellballen" pattern with an appearance similar to glomus tumor. Grimelius stain showed dark-brown secretory granules (Fig. 4) in the cytoplasm of the tumor cells. Neuron-specific enolase (NSE) stain was positive (Fig. 5), suggesting the presence of neuroendocrine cells. Other stains, including PAS, Chromogranin, and S-lOO protein, were all negative. The electron micrograph of glutalaldehyde-fixed tumor shown in Fig. 6 reveals the electron-dense granules (l00-150 nm). DISCUSSION
Paragangliomas ansmg in the head and neck are uncommon. The most common types are carotid body tumor, glomus tympanicum tumor, and glomus jugulare tumor (synonym: jugulotympanic paraganglioma). With respect to the site of origin of primary nasopharyngeal paraganglioma, GIRGIS and FAHMY (1973) speculated that paraganglionic tissue is normally present at birth surrounding the terminal part of the maxillary artery in the pterygopalatine fossa, and they suggested that the term "glomus nasopharyngis" should be applied to this group of tumors. Prior to this report, several nasopharyngeal paragangliomas arising in nearby paraganglia (e.g. jugulotympanic paraganglion, nodose paraganglion, and ciliary paraganglion) with possible extension into the nasopharynx were reported (SCOPPA and TONKIN, 1975; LACK, CUBILLA, WOODRUFF, and FARR, 1977; SCHULLER and LUCAS, 1982; CHAMBERS, NORMAN, DEDO, and FERRELL, 1982; KUHN and ARONOFF, 1989). Four cases of jugulotympanic paragangliomas were reported to
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
311
Fig. 6. Electron micrograph of the glutalaldehyde-fixed tumor demonstrating a small number of electron-dense granules (100-150 nm), suggesting the presence of neurosecretory granules.
312
N. KANOH et al.
exhibit the most aggressive local invasion (ASH and RAUM, 1949; WEILLE and LANE, 1951; DILL, 1959). Two nasopharyngeal paragangliomas originating from the nodose paraganglion origin have been reported by HOUSE et al. (1972). SMITH et al. (1973) described one case of ciliary ganglion origin. Taking into account the speculation of GIRGIS and FAHMY, as well as the location of the tumor, we suspect that the present tumor originated from the pterygopalatine ganglion, and thus can be classified as "glomus nasopharyngis." We propose that this kind of tumor should be termed "primary nasopharyngeal paraganglioma" to distinguish it from the nasopharyngeal extension of tumors arising from nearby structures. The differential diagnosis between carcinoid tumor and paraganglioma is difficult (BARNES and PEEL, 1990). Paragangliomas exhibit the pathological features of typical Zellballen pattern, whereas carcinoid tumors show a cordlike or trabecular pattern. In this case, an initial diagnosis of carcinoid tumor was made from the biopsy specimen. However, the final diagnosis of paraganglioma was made from examination of the surgically resected specimen, which showed typical cell nests and the Zellballen pattern using H&E and several other staining methods and electron micoscopy. Both carcinoid tumor and paraganglioma are considered to be functioning tumors; however in this case the clinical symptoms and signs and results of blood and urine tests were within normal limits. The previously reported cases of nasopharyngeal paragangliomas demonstrated no clinical functioning activity, with the exception of three recent cases (MATISHAK, SYMON, CHEESEMAN, and PAMPHLETT, 1987; OTOKlDA et al., 1987; OZAKURA et al., 1988). Whole-body MIBG (I-meta-iodobenzyl-guanidine) scintigraphy has been reported to be a useful method for detection of the primary lesion of catecholamine-secreting tumors (NAKAJO, SHAPIRO, Copp, KALFF, GROSS, SISSON, and BEIERWALTES, 1983). However, OZAKURA et al. (1988) reported that it was only successful in detecting the primary lesion in one case. The primary lesions in the other two cases were detected by performing selective venous blood collections and measuring for catecholamine concentrations. The only curative treatment for paraganglioma is adequate surgical excision of the tumor together with adjacent normal tissues. SCOPPA and TONKIN (1975) reported one case in which hemorrhage was marked and difficult to control during surgery. In order to achieve complete extirpation with detection of the feeding artery and minimization of blood loss, the three recent cases with large tumors underwent angiography and feeding artery embolization prior to surgery (OTOKIDA et al., 1987; MATISHAK et al., 1987; OZAKURA et al., 1988). New operative techniques, such as laser surgery and cryosurgery were also applied for tumor resection and control of bleeding. At least seven patients received irradiation therapy (ZAK and LAWSON, 1982). Chemotherapy was also administered in two cases (ZAK and LAWSON, 1982;
PRIMARY NASOPHARYNGEAL PARAGANGLIOMA
313
MORIMOTO et al. 1985). Morimoto achieved moderate results with chemotherapy, however, this was temporary. Fortunately, the tumor in our patient was detected at an early stage, and total resection could be performed without any complications. At present, neither postoperative irradiation nor chemotherapy is indicated, and careful follow-up examinations are regularly conducted. NSE stain in the present case was strongly positive. The NSE value of peripheral blood may be a good indicator of recurrence. CONCLUSION
We reported a rare case of nasopharyngeal paraganglioma with no clinical functioning activity. According to the literature, this was the fourth case in Japan, and the twentieth case in the world. To distinguish paraganglioma from nasopharyngeal extension arising from a nearby paraganglion, we term paragangliomas arising from the pterygopalatine ganglion "primary nasopharyngeal paraganglioma." We fortunately detected this tumor at an early stage, and were able to perform complete resection. This case emphasizes the importance of routine ENT examinations, especially in patients with abnormal findings in the nasopharynx. We are grateful for the kind advice of Prof. Takeo Kumoi and the English proofreading of Dr. Wendy Gray.
REFERENCES ASH, J. E., and RAUM, M.: An Atlas of Otolaryngic Pathology, 4th ed., Armed Forces Institute of Pathology, Washington, D.C., 1949. BARNES, L., and PEEL, R. L.: Head and Neck Pathology: A Text! Atlas of Differential Diagnosis, Chap 21: Paraganglioma versus carcinoid, pp. 45-46, Igaku-shoin, New York-Tokyo, 1990. CHAMBERS, E. F., NORMAN, D., DEDO, H. H., and FERRELL, L. D.: Primary nasopharyngeal chemodectoma. Neuroradiology 23: 285-288, 1982. DILL, J. L.: Tumors of the glomus jugular is. A report of nine cases. Laryngoscope 69: 248259, 1959. GIRGIS, 1. H., and FAHMY, S. A.: Nasopharyngeal fibroma; its histopathological nature. J. Ololaryngol. 0101. 87: 1107-1123, 1973. HOUSE, J. M., GOODMAN, M. L., GACEK, R. R., and GREEN, G. L.: Chemodectomas of the nasopharynx. Arch. Ololaryngol. 96: 138-141, 1972. KUHN, J. A., and ARONOFF, B. L.: Nasal and Nasopharyngeal Paraganglioma. J. Surg. Oncol. 40: 38-45, 1989. LACK, E. E., CUBILLA, A. L., WOODRUFF, J. M., and FARR, H. W.: Paragangliomas of the head and neck region. Cancer 39: 397-409, 1977. MATISHAK, M. Z., SYMON, L., CHEESEMAN, A., and PAMPHLETT, R.: Catecholamine-secreting paragangliomas of the base of the skull-Report of two cases. J. Neurosurg. 66: 604-608, 1987.
N. KANOH ef al.
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MORAN, T. E.: Non-chromaffin paraganglioma of the nasal cavity. Laryngoscope 72: 201206,1962. MORIMOTO, K., MATSUMOTO, K ., YOSHIMINE, T., and MOGAMI, H.: Nasopharyngeal Paraganglioma extended to middle cranial fossa. Neurosurgery (in Japanese) 13, 805-809, 1985. NAKAJO, M., SHAPIRO, B., Copp, J., KALFF, V., GROSS, M. D., SISSON, J. C., and BEIERWALTES, W. H.: The norm~1 and abnormal distribution of the adrenomedullary imaging agent m-[I-1311 iodobenzylguanidine (1-131 MlBG) in man: Evaluation by scintigraphy. J. Nllc/. Med. 24: 672-682, 1983. OTOKIDA, K ., OHlRA, A., KAMIMURA, A., FUJIMORI, M ., KATO, M., TAKAHASHI, A ., SAIKI, I., KANAYA, H., TASHIRO, A., and ABE, T.: Cervical catecholamine- secreting paraganglioma in the pterygopalatine fossa. Tohokll J. Exp. Med. 153,347-354, 1987. OZAKURA, K., KIMURA, T., and KISHIMOTO, S. : Primary pterygopalatine catecholamine-secreting paraganglioma. H ead Neck Cancel' Jpn. (in Japanese) 15: 57, 1988. PARISIER, S. c., and SINCLAIR, G . M.: Glomus tumor of the nasal cavity. Laryngoscope 78: 2013-2024, 1968. PEREZ, C. A., ACKERMAN, L. V., and MILL, W. B.: Cancer of the nasopharynx: Factors influencing prognosis. Cancel' 24: 1-17, 1969. SCHULLER, D. E., and LUCAS, J. G . : Nasopharyngeal Paraganglioma- Report of a case and review of Iiterature- . Arch. 0 101. 108: 667-670, 1982. SCOPPA, J., and TONKIN, J . P.: Non-chromaffin paraganglioma of the nasopharynx. J . Laryngol. 89: 653-656, 1975. SMITH, C. J., KOHUT, R. r., and MILLION, R. R . : Chemodectomas of the nasopharynx. Laryngoscope 83: 330-335, 1973. WEILLE, F. L., and LANE, C. S.: Surgical problems involved in the removal of glomus-jugulare tumors. Laryngoscope 61, 448- 459, 1951. ZAK, F. G., and LAWSON, W. : Tumors of the Nose and Nasopharynx. The Paraganglionic Chemoreceptor System, pp. 276-285, Springer-Verlag, New York, 1982.
Request reprints to:
Dr. N. Kanoh, Department of Otolaryngology, Hyogo College of Medicine, I-I Mukogawa-cho, Nishinomiya 663, Japan
