Auris·Naslls·Larynx (Tokyo) 18,33-38 (1991)
NEUROFIBROMA OF THE HYPOPHARYNX: A CASE REPORT Hiroshi MIYAHARA, M.D., Kunitoshi YOSHINO, M.D.,* and Takeo SATO, M .D .* DepartmeJl1 0/ Otolaryngology, Nara Medical University, Nara, Japan *Department 0/ Otolaryngology, The Cellfer /01' Adult Diseases, Osaka, Japan
A 45-year-old female patient with a neurofibroma of the hypopharynx is reported. She had complained of swallowing discomfort for 3 months. Indirect laryngoscopy showed an oval tumor with a smooth surface in the posterior wall of the hypopharynx. Xeroradiography was effective for defining the tumor. Removal of the tumor was performed by an infrahyoid horizontal pharyngotomy under general anesthesia. The tumor had an indistinct capsule. The nerve from which the tumor originated was not clear. Histologically, the tumor was diagnosed as neurofibroma. The patient had no other neurogenic tumor and has been well without recurrence after the surgery. Neurogenic tumors of the head and neck region are relatively rare. Classification of neurogenic tumors arising from Schwann cells has not yt.t been conclusive. Benign neurogenic tumors are generally diagnosed as neurinoma, neurilemmoma, neurofibroma, Schwannoma, etc. Neurinoma was first described by VEROCAY (1908). The cellular origin of neurofibroma has been discussed microscopically and biochemically. Recently we surgically treated a case of neurofibroma on the posterior wall of the hypopharynx. The case is reported with a review of related literature. CASE REPORT
A 45-year-old female consulted our hospital with a complaint of swallowing discomfort without pain for 3-month duration. Indirect laryngoscopy revealed an oval exophytic tumor on the posterior wall of the hypopharynx with a relatively wide base. The surface of the tumor was smooth and covered with mucosa. Flexible fiberscopy through the nasal cavity also revealed an oval tumor Received for publication
May 10, 1990 33
34
H. MIYAHARA, K. YOSHINO, and T. SATO
Fig. 1. Afiberscopic view of the tumor on the posterior wall of the hypopharynx.
Fig. 3.
A radiographic view (P-A view).
Fig. 2. A radiographic view (barium swallow) showing soft tissue mass (lateral view) .
Fig. 4. A xeroradiographic view showing the mass (lateral view).
(Fig. 1). The tumor appeared to be a benign tumor of the hypopharynx. Biopsy was' attempted under indirect laryngoscopy but failed . Laryngopharyngography showed a round, smooth tumor on the posterior wall of the hypopharynx (Figs. 2 and 3). Lateral xeroradiography clearly demonstrat-
NEUROFIBROMA OF THE HYPOPHARYNX
35
ed a contour of the tumor on the posterior wall of the hypopharynx (Fig. 4). Although the tumor was situated in the supralaryngeal region, either dyspnea or dysphagia were not complained of. Physical examination revealed no other lesions. She was hospitalized, and on September 27, 1981 operated on under general anesthesia following tracheotomy. A horizontal skin incision of approximately 5 cm long was made at the level of the hyoid bone. By infrahyoid pharyngotomy the preepiglottic space was cut through. When the pharyngeal space was opened, a good exposure was obtained and the epiglottis and the tumor were observed. The tumor was covered with pharyngeal mucosa with a relatively wide base (Fig. 5). The tumor had no distinct capsule and was removed. After hemostasis, the pharyngeal mucosa was primarily closed. The skin incision was closed with 4-0silk. A feeding tube was inserted. The tumor was 22 x 18 x 16 mm in size and 25 g in weight, and it appeared to be non-encapsulated (Fig. 6). The cut surface was whitish gray in color (Fig. 7). Histopathological examination showed a wavy, elongated, spindle cell pattern of neurofibroma comprised mainly of elongated fibroblast-like cells (Fig. 8). The
Fig. 5.
Fig. 6.
The specimen.
The tumor at operation.
Fig. 7.
The specimen divided.
36
Fig. 8.
H. MIYAHARA, K. YOSHINO, and T. SATO
Photomicroscopy of the tumor shows spindle shaped cells with wavy pattern ( x 100).
tracheal cannula was removed on the 4th postoperative day, oral feeding was started 5 days after the operation, and the patient discharged on the 14th day. Eight years later, she had no recurrence of the tumor . DISCUSSION
Neurogenic tumors are relatively rare in the head and neck region, and most of them are benign. Histologically, benign neurogenic tumors are divided into neurilemmomas, neurofibromas, and ganglioneuromas. VEROCAY (1908) first described tumors originating from the nerve sheath, called neurinoma. In 1935, STOUT described cases of neurilemmoma occurring in many sites of the human body. He proposed that neurilemmomas arise from the sheath of Schwann of the peripheral nerves. Terminology of benign tumors of nerve sheath origin are still confusing: neurilemmoma, neurinoma, Schwannoma, and neurofibroma are used . STRAUS and GUCKIEN (1951) discussed neurofibromas and neurilemmomas (neurinoma, peripheral fibroblastoma, Schwannoma, peripheral glioma, and Schwannoglioma). Electron microscopically, PUTNEY, MORAN, and THOMAS (1964), ISHII and KAWABATA (1968), and GRAVIOTO (1968) showed that neurilemmomas • and neurofibromas are originated from Schwann cells. In other words, Schwann cells can be the parent cells of both types. We reported the ultrafine structure of neurilemmomas in the parapharyngeal space (MIYAHARA, OHBUCHI, and SATO, 1977). The tumor cells were surrounded by a continuous basal lamina and well defined. In the cytoplasm, fine filaments were observed. Clinical and histological differences among neurilemmomas, neurinomas, neurilemmomata, and neurofibromas and neurofibromata are summarized in Table l. In the present case, the tumor was solitary and non-encapsulated. Histologically, elongated cells were arranged in parallel, in a wavy pattern. Nucleoli
NEUROFIBROMA OF THE HYPOPHARYNX Table I .
37
Clinical features of neurilemmomata and neurofibromata.
Neurilemmoma ta
Neurofibromata
I. 2. 3. 4.
Solitary Encapsulated Attached to , or surrounded by, a nerve Almost never associated with Von Recklinghausen's disease 5. Almost never undergo malignant change
5.
6. 7. 8. 9.
6. 7. 8. 9.
Neur ites do not traverse the tumor Degenerative changes are usually present Centrifugal distribution Often painful
I. 2. 3. 4.
Usually multiple Non-encapsulated Occur within the nerve sheath Often associated with Von Recklinghausen's disease Approximately 12 % undergo malignant change Neurites pass through the tumor Retrogressive changes are less common Centripetal distribution Uncommonly painful
By GOODER and FARRINGTON (1980).
were clear and also elongated. The last four cranial nerves and the cervical sympathetic chain are usually the sites of origin of tumors in the neck. The nerve of origin was not clear in our case. The tumor of this case occurred on the posterior wall of the hypopharynx. While neurogenic tumors including neurilemmoma and neurofibroma in the head and neck are uncommon, most of them may occur in the lateral cervical region (GUPTA, BRASFIELD, STRONG, and HAJDU, 1969). Presenting in the pharynx, chiefly the parapharyngeal space and the larynx is rare (NANSON, 1978; GOODER and FARRINGTON, 1980). There were no reports of neurofibroma in the hypopharynx. In our case, a relatively large tumor was observed over the epiglottis without dyspnea. The tumor was clearly shown in the lateral view by pharyngography and xeroradiography. Pharyngography is useful for estimating the size and extent of the tumor. Xeroradiography is also more effective in defining a tumor. The approach to removal can be divided into two: an infrahyoid approach and a suprahyoid approach. We operated by the infrahyoid approach, with good control of bleeding and care not to injure the superior laryngeal artery, nerves, or lingual artery. This approach is the safest and most appropriate method. REFERENCES GOODER, P., and FARRINGTON, T .: Extracranial neurilemmomata of the head and neck. 1. Laryl/gol. Otol. 94: 243- 249, 1980. GRAYIOTO, H. : Long-spacing fibrous collagen in human acoustic nerve tumors. /1/ vil'o and ill vitro observations. 1. Ultrastruct . Res. 24 : 70-85, 1968. GUPTA, T. K., BRASFIELD, R .D., STRONG, E. W., and HAJDU, S. 1. : Benign solitary Schwannoma (neurilemmomas). Callcer 24: 355-366, 1969. ISHII, H., and KAWABATA, I.: Ultrastructure of the neurilemmoma. Pract. Otol. (Kyoto) (in Japanese) 61: 231-242,1968. MIYAHARA, H., OHBUCHI, M., and SATO, T.: Electron microscopic observations of neurilemmoma in parapharyngea l space. 1. Clill. Electron Microsc. 10: 637-638, 1977.
38
H. MIYAHARA, K. YOSHINO, and T. SATO
NANSON, E. M.: Neurilemmoma of the larynx: A case study. Head Neck Surg. 1: 69-74, 1978. PUTNEY, F. J., MORAN, J. J., and THOMAS, G. K.: Neurogenic tumors of the head and neck. Laryngoscope 74: 1037-1059, 1964. STOUT, A. P.: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma). Am. J. Cancer 24: 751-796, 1935. STRAUS, G. D., and GUCKIEN, J. L.: Schwan noma of the tracheobronchial tree. Ann. Otol. Rhinol. Laryngol. 60: 242-246, 1951. VEROCAY, J.: Multiple Geschwulste als Systemerkrankung am nervosen Apparate, pp. 378415, Festschrift fUr Chiari, Wien und Leipzig, W. Brauriuller, 1908.
Request reprints to:
Dr. H. Miyahara, Department of Otolaryngology, Nara Medical University, 840 Shijocho, Kashihara, Nara 634, Japan
NEUROFIBROMA OF THE HYPOPHARYNX: A CASE REPORT Hiroshi MIYAHARA, M.D., Kunitoshi YOSHINO, M.D.,* and Takeo SATO, M .D .* DepartmeJl1 0/ Otolaryngology, Nara Medical University, Nara, Japan *Department 0/ Otolaryngology, The Cellfer /01' Adult Diseases, Osaka, Japan
A 45-year-old female patient with a neurofibroma of the hypopharynx is reported. She had complained of swallowing discomfort for 3 months. Indirect laryngoscopy showed an oval tumor with a smooth surface in the posterior wall of the hypopharynx. Xeroradiography was effective for defining the tumor. Removal of the tumor was performed by an infrahyoid horizontal pharyngotomy under general anesthesia. The tumor had an indistinct capsule. The nerve from which the tumor originated was not clear. Histologically, the tumor was diagnosed as neurofibroma. The patient had no other neurogenic tumor and has been well without recurrence after the surgery. Neurogenic tumors of the head and neck region are relatively rare. Classification of neurogenic tumors arising from Schwann cells has not yt.t been conclusive. Benign neurogenic tumors are generally diagnosed as neurinoma, neurilemmoma, neurofibroma, Schwannoma, etc. Neurinoma was first described by VEROCAY (1908). The cellular origin of neurofibroma has been discussed microscopically and biochemically. Recently we surgically treated a case of neurofibroma on the posterior wall of the hypopharynx. The case is reported with a review of related literature. CASE REPORT
A 45-year-old female consulted our hospital with a complaint of swallowing discomfort without pain for 3-month duration. Indirect laryngoscopy revealed an oval exophytic tumor on the posterior wall of the hypopharynx with a relatively wide base. The surface of the tumor was smooth and covered with mucosa. Flexible fiberscopy through the nasal cavity also revealed an oval tumor Received for publication
May 10, 1990 33
34
H. MIYAHARA, K. YOSHINO, and T. SATO
Fig. 1. Afiberscopic view of the tumor on the posterior wall of the hypopharynx.
Fig. 3.
A radiographic view (P-A view).
Fig. 2. A radiographic view (barium swallow) showing soft tissue mass (lateral view) .
Fig. 4. A xeroradiographic view showing the mass (lateral view).
(Fig. 1). The tumor appeared to be a benign tumor of the hypopharynx. Biopsy was' attempted under indirect laryngoscopy but failed . Laryngopharyngography showed a round, smooth tumor on the posterior wall of the hypopharynx (Figs. 2 and 3). Lateral xeroradiography clearly demonstrat-
NEUROFIBROMA OF THE HYPOPHARYNX
35
ed a contour of the tumor on the posterior wall of the hypopharynx (Fig. 4). Although the tumor was situated in the supralaryngeal region, either dyspnea or dysphagia were not complained of. Physical examination revealed no other lesions. She was hospitalized, and on September 27, 1981 operated on under general anesthesia following tracheotomy. A horizontal skin incision of approximately 5 cm long was made at the level of the hyoid bone. By infrahyoid pharyngotomy the preepiglottic space was cut through. When the pharyngeal space was opened, a good exposure was obtained and the epiglottis and the tumor were observed. The tumor was covered with pharyngeal mucosa with a relatively wide base (Fig. 5). The tumor had no distinct capsule and was removed. After hemostasis, the pharyngeal mucosa was primarily closed. The skin incision was closed with 4-0silk. A feeding tube was inserted. The tumor was 22 x 18 x 16 mm in size and 25 g in weight, and it appeared to be non-encapsulated (Fig. 6). The cut surface was whitish gray in color (Fig. 7). Histopathological examination showed a wavy, elongated, spindle cell pattern of neurofibroma comprised mainly of elongated fibroblast-like cells (Fig. 8). The
Fig. 5.
Fig. 6.
The specimen.
The tumor at operation.
Fig. 7.
The specimen divided.
36
Fig. 8.
H. MIYAHARA, K. YOSHINO, and T. SATO
Photomicroscopy of the tumor shows spindle shaped cells with wavy pattern ( x 100).
tracheal cannula was removed on the 4th postoperative day, oral feeding was started 5 days after the operation, and the patient discharged on the 14th day. Eight years later, she had no recurrence of the tumor . DISCUSSION
Neurogenic tumors are relatively rare in the head and neck region, and most of them are benign. Histologically, benign neurogenic tumors are divided into neurilemmomas, neurofibromas, and ganglioneuromas. VEROCAY (1908) first described tumors originating from the nerve sheath, called neurinoma. In 1935, STOUT described cases of neurilemmoma occurring in many sites of the human body. He proposed that neurilemmomas arise from the sheath of Schwann of the peripheral nerves. Terminology of benign tumors of nerve sheath origin are still confusing: neurilemmoma, neurinoma, Schwannoma, and neurofibroma are used . STRAUS and GUCKIEN (1951) discussed neurofibromas and neurilemmomas (neurinoma, peripheral fibroblastoma, Schwannoma, peripheral glioma, and Schwannoglioma). Electron microscopically, PUTNEY, MORAN, and THOMAS (1964), ISHII and KAWABATA (1968), and GRAVIOTO (1968) showed that neurilemmomas • and neurofibromas are originated from Schwann cells. In other words, Schwann cells can be the parent cells of both types. We reported the ultrafine structure of neurilemmomas in the parapharyngeal space (MIYAHARA, OHBUCHI, and SATO, 1977). The tumor cells were surrounded by a continuous basal lamina and well defined. In the cytoplasm, fine filaments were observed. Clinical and histological differences among neurilemmomas, neurinomas, neurilemmomata, and neurofibromas and neurofibromata are summarized in Table l. In the present case, the tumor was solitary and non-encapsulated. Histologically, elongated cells were arranged in parallel, in a wavy pattern. Nucleoli
NEUROFIBROMA OF THE HYPOPHARYNX Table I .
37
Clinical features of neurilemmomata and neurofibromata.
Neurilemmoma ta
Neurofibromata
I. 2. 3. 4.
Solitary Encapsulated Attached to , or surrounded by, a nerve Almost never associated with Von Recklinghausen's disease 5. Almost never undergo malignant change
5.
6. 7. 8. 9.
6. 7. 8. 9.
Neur ites do not traverse the tumor Degenerative changes are usually present Centrifugal distribution Often painful
I. 2. 3. 4.
Usually multiple Non-encapsulated Occur within the nerve sheath Often associated with Von Recklinghausen's disease Approximately 12 % undergo malignant change Neurites pass through the tumor Retrogressive changes are less common Centripetal distribution Uncommonly painful
By GOODER and FARRINGTON (1980).
were clear and also elongated. The last four cranial nerves and the cervical sympathetic chain are usually the sites of origin of tumors in the neck. The nerve of origin was not clear in our case. The tumor of this case occurred on the posterior wall of the hypopharynx. While neurogenic tumors including neurilemmoma and neurofibroma in the head and neck are uncommon, most of them may occur in the lateral cervical region (GUPTA, BRASFIELD, STRONG, and HAJDU, 1969). Presenting in the pharynx, chiefly the parapharyngeal space and the larynx is rare (NANSON, 1978; GOODER and FARRINGTON, 1980). There were no reports of neurofibroma in the hypopharynx. In our case, a relatively large tumor was observed over the epiglottis without dyspnea. The tumor was clearly shown in the lateral view by pharyngography and xeroradiography. Pharyngography is useful for estimating the size and extent of the tumor. Xeroradiography is also more effective in defining a tumor. The approach to removal can be divided into two: an infrahyoid approach and a suprahyoid approach. We operated by the infrahyoid approach, with good control of bleeding and care not to injure the superior laryngeal artery, nerves, or lingual artery. This approach is the safest and most appropriate method. REFERENCES GOODER, P., and FARRINGTON, T .: Extracranial neurilemmomata of the head and neck. 1. Laryl/gol. Otol. 94: 243- 249, 1980. GRAYIOTO, H. : Long-spacing fibrous collagen in human acoustic nerve tumors. /1/ vil'o and ill vitro observations. 1. Ultrastruct . Res. 24 : 70-85, 1968. GUPTA, T. K., BRASFIELD, R .D., STRONG, E. W., and HAJDU, S. 1. : Benign solitary Schwannoma (neurilemmomas). Callcer 24: 355-366, 1969. ISHII, H., and KAWABATA, I.: Ultrastructure of the neurilemmoma. Pract. Otol. (Kyoto) (in Japanese) 61: 231-242,1968. MIYAHARA, H., OHBUCHI, M., and SATO, T.: Electron microscopic observations of neurilemmoma in parapharyngea l space. 1. Clill. Electron Microsc. 10: 637-638, 1977.
38
H. MIYAHARA, K. YOSHINO, and T. SATO
NANSON, E. M.: Neurilemmoma of the larynx: A case study. Head Neck Surg. 1: 69-74, 1978. PUTNEY, F. J., MORAN, J. J., and THOMAS, G. K.: Neurogenic tumors of the head and neck. Laryngoscope 74: 1037-1059, 1964. STOUT, A. P.: The peripheral manifestations of the specific nerve sheath tumor (neurilemmoma). Am. J. Cancer 24: 751-796, 1935. STRAUS, G. D., and GUCKIEN, J. L.: Schwan noma of the tracheobronchial tree. Ann. Otol. Rhinol. Laryngol. 60: 242-246, 1951. VEROCAY, J.: Multiple Geschwulste als Systemerkrankung am nervosen Apparate, pp. 378415, Festschrift fUr Chiari, Wien und Leipzig, W. Brauriuller, 1908.
Request reprints to:
Dr. H. Miyahara, Department of Otolaryngology, Nara Medical University, 840 Shijocho, Kashihara, Nara 634, Japan
