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Malignant teratocarcinosarcoma of the sphenoid sinus
Malignant teratocarcinosarcoma of the sphenoid sinus LARRY SHEMEN, MD, FRCS, FACS, PETER GALANTICH, MD, and RAJ MURALI, MD, FRCS, FACS,
New York and Pompton Plains, New York
All I¥1alignant t e r a t o c a r c i n o s a r c o m a is a rare neoplastic entity u n c o m m o n in the h e a d and neck. It has most often b e e n described as originating in the nose and paranasal sinuses. T h e diversity of histologic patterns has led it to be classified u n d e r m a n y designations. T h e t e r m malignant teratocarcinosarcoma was coined by H e f f n e r and H y a m s ~ in an a t t e m p t to unify the various diagnoses. T h e first d o c u m e n t e d case of this rare neoplastic entity originating in the sphenoid sinus will be discussed. T h e surgical a p p r o a c h with c o m p l e t e r h i n o t o m y and bilateral medial maxillectomy for skull base exposure will be examined. CASE REPORT
A 46-year-old man sought treatment with a 6-week
history of bilateral nasal obstruction; pain and numbness involving the right cheek, right nose, and right upper lip; diplopia; inability to focus his eyes; right temporal and occipital headaches; and left-sided epistaxis. He had been treated with topical and systemic decongestants with little relief. Past medical history was otherwise unremarkable. He smoked 1.5 packs of cigarettes per day but denied aleohol intake. Physieal examination disclosed a well-nourished, obese man. The right eyelid was ptotic with a right oculomotor nerve palsy and a moderately dilated pupil. The fundoscopic examination was normal. Intranasal examination revealed a bilateral, white mass originating superiorly. There was hypesthesia to pain stimuli in the right V1 and V2 distribution. T h e remaining head and neck and neurologic examinations were normal. Computed tomography (CT) and magnetic resonance
From the Department of Head and Neck Surgery (Dr. Shemen), Manhattan Eye, Ear and Throat Hospital; Chilton Memorial Hospital (Dr. Galantich); and the Department of Neurosurgery (Dr. Murali), St. Vincent's Hospital and Medical Center of New York. Received for publication May 24, 1994; Sept. 30, 1994. Reprint requests: Larry Shemen, MD, FRCS, FACS, 233 E. 69 St., New York, NY 10021. OTOLARYNGOLHEADNEcKSIJRa 1995;112:496-500. Copyright © 1995 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/95/$3.00 + 0 23/4/60942 496
Fig. I. Preoperative CT, coronal cut bone window showing sellar erosion.
imaging (MRI) scans were obtained, and these showed a large tumor with speckled calcification originating in the sphenoid sinus and expanding into the posterior ethmoid cells. The tumor eroded the tuberculum sella and displaced the cavernous sinus laterally to the right. The tumor abutted the optic chiasm but did not displaee it. No erosion of the dura was demonstrated (Figs. 1 and 2). A biopsy was performed, and this was interpreted as infiltrating craniopharyngioma. In view of this tumor's aggressive behavior, it was considered a malignant craniopharyngioma, and a combined craniofacial approach on Sept. 20, 1988, was planned. A standard Weber-Fergusson incision was made on the right side but carried across the nasal root to the left side (Fig. 3). Bilateral lateral osteotomies were performed with extension across the nasion. Drill holes were placed through the nasal bones and ascending processes of the maxilla for later closure. A transfixion incision was made through the septum, and an elevation of the septum over the maxillary crest allowed the nose to be reflected onto the left cheek. At this stage, bilateral medial maxillectomies were performed with a standard technique. Mobilization of the septum allowed for an unobstructed view of the sphenoid sinus. Bilateral ethmoidectomies were per-
Otolaryngolocjy Head
and
Surgery Number 3
Neck
Volume 1t2
$HEMEN et aL
497
Fig. 2. Preoperative CT, coronal out soff-tissue window demonstrating expansion into the antra and nasal cavities.
Fig. 4. Completed rhinotomy and bilateral medial maxilleotomies.
Fig. 5. Postoperative CT, axial cut bone window showing defect at 5 years.
Fig. 3. Markings for incisions showing Weber-Fergusson incision with extension across nasal root witn modified Lynch incision to allow for complete rhinotomy with pedicling of the nose on facial skin flap.
formed, and the tumor was resected from within the ethmoids (Fig. 4). As the sphenoid was entered, it was apparent that no bone remained along the superior, lateral, or inferior walls. Dura was exposed along the wall posterior to the clinoid process but was not invaded by the tumor. With the use of the operating microscope, the remainder of the tumor was meticulously dissected from
498
SHEMEN et af.
Otolaryngology Head and Neck Surgery March 1995
Fig. 6. Postoperative CT, coronal cut bone window showing defect at 5 years.
Fig. 8. Postoperative photograph at 5 years.
Fig. 7ù Postoperative CT, coronal cut soft-tissue window, showing defect at 5 years.
the dura by use of ring curettes. Tumor was traced to the cavernous sinus, but this structure was compressed rather than invaded by the tumor, and it was therefore dissected away with no dural penetration. The pituitary gland and optic chiasm were suspended in tumor, and these were also dissected clean. The tumor was thus completely resected through the complete rhinotomy and transethmoid/transsphenoid approach. After the resection, the sphenoid sinus was packed, as were the maxillary sinuses. The nasal bones were wired into apposition. The medial canthal ligaments were reattached. The postoperative course was uneventful. The diplopia and facial numbness improved. Postoperative radiotherapy (primary site: 61.2 Gy, anterior 6 × 9 cm and lateral 9 x 9 cm fields; bilateral necks: 50 Gy, 20 x 21 cm) was administered. Annual follow-up CT scans and MRI at 5 years failed to show any recurrence (Figs. 5, 6, and 7) of tumor, and the patient remains free of symptoms (Fig. 8). The tumor was sent to the Armed Forces Institute of Pathology for interpretation (Drs. Heffner, Durham, and Karnei). There were carcinomatous malignant areas that were mixed with some spindled and stromal areas with a sarcomatoid appearance (Fig. 9). In addition, there were foci of well-differentiated squamous tissue typical of benign tissue (Fig. 10). The final diagnosis, based on
Otolaryngoiogy Head and Neck Surgery Volume 112 Number 3
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Fig. 9. Low-power micrograph showing variegated appearance. (Hematoxylin and eosin stain;
x 100.)
Fig. t0. High-power micrograph showing spindling and sarcomatoid appearance. (Hematoxytin and eosin stain; × 250.)
these variegated components, was malignant teratocarcinosarcoma. DISCUSSION
The term malignant teratocarcinosarcoma was derived by Heffner and Hyams I to describe a rare tumor of the sinonasal tract. Various other terms have been used to describe this tumor, including carcinosarcoma, malignant teratoma, and teratocarcinoma. The variegated histologic pattern was used to distinguish this tumor as a separate disease entity.
The histologic diagnosis is based on a combination of epithelial and connective tissue elements, such as fibroblasts, cartilage, bone, and, smooth muscle. If only a single mesenchymal component is present, the lesion is termed a carcinosarcoma. 2 Tissue heterogeneity and a variegated architectural pattern are characteristic of these teratoid lesions? The tumor is thought to originate from immature pleuripotential cells that have become sequestered in the sinonasal tract. Small samples, such as from biopsies, may not show the true histologic range of these tumors. This may account for the original biopsy
500
Otolaryngology Head and Neck Surgery March 1995
SHEMEN et al.
diagnosis in our case of malignant craniopharyngioma. Typical symptoms include nasal obstruction and epistaxis. Examination shows a polypoid mass. CT demonstrates opacification of one or several sinuses with bone destruction. These tumors are locally aggressive and may invade the orbit or cranium. In 6 (30%) of 20 cases cervical nodal metastasis was present or developed. Follow-up information was available for 17 cases: 8 had recurrence, 9 died with disease, and 1 was alive with disease. Five patients had no evidence of disease as long as 9 years later, and one died of an unrelated condition and was disease free at the time of death (30% 5-year survival). We reviewed six other cases in the literature with similar results. The recommended treatment for this condition was surgery and radiation therapy. Shanmugaratnam et al? reviewed three additional cases and also advocated postoperative radiation therapy. Devgan et al., 5 Christensen et al., 6 and Martinez et al. 7 each reviewed an additional case. Of the 20 cases reviewed by Heffner and Hyams in 1984,1 none originated primarily in the sphenoid sinus. Sites of origin have been described as the nasal cavity, maxillary sinus, ethmoid sinus, posterior choana, and orbit. Tumors were found in the sphenoid sinus, but these were thought to have been extensions from surrounding ethmoid disease. The CT and MRI scans, as well as the intraoperative findings, clearly show that the tumor presented here originated in the sphenoid sinus. A review of the literature shows no cases originating in the sphenoid sinus. The anatomy of the sphenoid sinus makes the diagnosis of disease arising in this particular sinus different from those in the other sinuses. The sphenoid begins to develop during the third year of life from an invagination of the sphenoethmoidal recess. It reaches adult size late in the second decade of life. As the sphenoid expands, it abuts adjacent neurologic and vascular structures. The optic nerves, carotid artery, and vidian nerves all lie in close proximity. In our case, the tumor exl~anded laterally to the right affecting the ophthalmic and maxillary branches of the trigeminal nerve and oculomotor
nerve. The tumor had eroded up to the optic chiasm. This, however, did not affect the patient's visual acuity. The return of normal sensation and motor function several weeks after surgery are consistent with a neuropraxic type of injury. The approach used for the surgical resection of this tumor offered maximum exposure of the sphenoid sinus. The complete rhinotomy and bilateral maxillectomy used in conjunction with a transsphenoid/transethmoid approach allowed for complete resection of the tumor with minimal morbidity. In addition, there was minimal disfigurement. \
SUMMARY
We have presented the first reported case of malignant teratocarcinosarcoma arising in the sphenoid sinus treated successfully with surgery and radiation therapy. The patient shows no signs of recurrence 5 years after surgery. The combined head and neck/neurosurgical approach using complete rhinotomy and medial maxillectomy with transsphenoid/transethmoid approach is advocated for sphenoid sinus tumors of this type. We would like to acknowledge Dede Silverstone for library assistance and Winston Harrison, MD, for the photomicrographs. REFERENCES
1. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer 1984;53:2140-54. 2. Shindo ML, Stanley RB, Kiyabu MT. Carcinosarcoma of the nasal cavity and paranasal sinuses. Head Neck 1990;12:516-9. 3. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GSC, Sinniah R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413-9. 4. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GSC, Sinniah R. Terataid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413-9. 5. Devgan BK, Devgan M, Gross CW. Teratocarcinoma of the sphenoid sinus: review of the literature plus a new case report. OTOLARYNGOLI-IEADNECKSURG1978;86:689-95. 6. Christensen EC, Fu YS, Wilson LJ, Hoover LA. Teratoid carcinosarcoma of the nasal and paranasal cavities: a case report. Am J Rhinol 1992;6:169-72. 7. Martinez R, Lopez R, Parra R, Rodriguez B. Sinusal teratocarcinosarcoma. Acta Otorrinolaringol Esp 1991;42:363-7.
New York and Pompton Plains, New York
All I¥1alignant t e r a t o c a r c i n o s a r c o m a is a rare neoplastic entity u n c o m m o n in the h e a d and neck. It has most often b e e n described as originating in the nose and paranasal sinuses. T h e diversity of histologic patterns has led it to be classified u n d e r m a n y designations. T h e t e r m malignant teratocarcinosarcoma was coined by H e f f n e r and H y a m s ~ in an a t t e m p t to unify the various diagnoses. T h e first d o c u m e n t e d case of this rare neoplastic entity originating in the sphenoid sinus will be discussed. T h e surgical a p p r o a c h with c o m p l e t e r h i n o t o m y and bilateral medial maxillectomy for skull base exposure will be examined. CASE REPORT
A 46-year-old man sought treatment with a 6-week
history of bilateral nasal obstruction; pain and numbness involving the right cheek, right nose, and right upper lip; diplopia; inability to focus his eyes; right temporal and occipital headaches; and left-sided epistaxis. He had been treated with topical and systemic decongestants with little relief. Past medical history was otherwise unremarkable. He smoked 1.5 packs of cigarettes per day but denied aleohol intake. Physieal examination disclosed a well-nourished, obese man. The right eyelid was ptotic with a right oculomotor nerve palsy and a moderately dilated pupil. The fundoscopic examination was normal. Intranasal examination revealed a bilateral, white mass originating superiorly. There was hypesthesia to pain stimuli in the right V1 and V2 distribution. T h e remaining head and neck and neurologic examinations were normal. Computed tomography (CT) and magnetic resonance
From the Department of Head and Neck Surgery (Dr. Shemen), Manhattan Eye, Ear and Throat Hospital; Chilton Memorial Hospital (Dr. Galantich); and the Department of Neurosurgery (Dr. Murali), St. Vincent's Hospital and Medical Center of New York. Received for publication May 24, 1994; Sept. 30, 1994. Reprint requests: Larry Shemen, MD, FRCS, FACS, 233 E. 69 St., New York, NY 10021. OTOLARYNGOLHEADNEcKSIJRa 1995;112:496-500. Copyright © 1995 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/95/$3.00 + 0 23/4/60942 496
Fig. I. Preoperative CT, coronal cut bone window showing sellar erosion.
imaging (MRI) scans were obtained, and these showed a large tumor with speckled calcification originating in the sphenoid sinus and expanding into the posterior ethmoid cells. The tumor eroded the tuberculum sella and displaced the cavernous sinus laterally to the right. The tumor abutted the optic chiasm but did not displaee it. No erosion of the dura was demonstrated (Figs. 1 and 2). A biopsy was performed, and this was interpreted as infiltrating craniopharyngioma. In view of this tumor's aggressive behavior, it was considered a malignant craniopharyngioma, and a combined craniofacial approach on Sept. 20, 1988, was planned. A standard Weber-Fergusson incision was made on the right side but carried across the nasal root to the left side (Fig. 3). Bilateral lateral osteotomies were performed with extension across the nasion. Drill holes were placed through the nasal bones and ascending processes of the maxilla for later closure. A transfixion incision was made through the septum, and an elevation of the septum over the maxillary crest allowed the nose to be reflected onto the left cheek. At this stage, bilateral medial maxillectomies were performed with a standard technique. Mobilization of the septum allowed for an unobstructed view of the sphenoid sinus. Bilateral ethmoidectomies were per-
Otolaryngolocjy Head
and
Surgery Number 3
Neck
Volume 1t2
$HEMEN et aL
497
Fig. 2. Preoperative CT, coronal out soff-tissue window demonstrating expansion into the antra and nasal cavities.
Fig. 4. Completed rhinotomy and bilateral medial maxilleotomies.
Fig. 5. Postoperative CT, axial cut bone window showing defect at 5 years.
Fig. 3. Markings for incisions showing Weber-Fergusson incision with extension across nasal root witn modified Lynch incision to allow for complete rhinotomy with pedicling of the nose on facial skin flap.
formed, and the tumor was resected from within the ethmoids (Fig. 4). As the sphenoid was entered, it was apparent that no bone remained along the superior, lateral, or inferior walls. Dura was exposed along the wall posterior to the clinoid process but was not invaded by the tumor. With the use of the operating microscope, the remainder of the tumor was meticulously dissected from
498
SHEMEN et af.
Otolaryngology Head and Neck Surgery March 1995
Fig. 6. Postoperative CT, coronal cut bone window showing defect at 5 years.
Fig. 8. Postoperative photograph at 5 years.
Fig. 7ù Postoperative CT, coronal cut soft-tissue window, showing defect at 5 years.
the dura by use of ring curettes. Tumor was traced to the cavernous sinus, but this structure was compressed rather than invaded by the tumor, and it was therefore dissected away with no dural penetration. The pituitary gland and optic chiasm were suspended in tumor, and these were also dissected clean. The tumor was thus completely resected through the complete rhinotomy and transethmoid/transsphenoid approach. After the resection, the sphenoid sinus was packed, as were the maxillary sinuses. The nasal bones were wired into apposition. The medial canthal ligaments were reattached. The postoperative course was uneventful. The diplopia and facial numbness improved. Postoperative radiotherapy (primary site: 61.2 Gy, anterior 6 × 9 cm and lateral 9 x 9 cm fields; bilateral necks: 50 Gy, 20 x 21 cm) was administered. Annual follow-up CT scans and MRI at 5 years failed to show any recurrence (Figs. 5, 6, and 7) of tumor, and the patient remains free of symptoms (Fig. 8). The tumor was sent to the Armed Forces Institute of Pathology for interpretation (Drs. Heffner, Durham, and Karnei). There were carcinomatous malignant areas that were mixed with some spindled and stromal areas with a sarcomatoid appearance (Fig. 9). In addition, there were foci of well-differentiated squamous tissue typical of benign tissue (Fig. 10). The final diagnosis, based on
Otolaryngoiogy Head and Neck Surgery Volume 112 Number 3
SHEMEN et al.
499
Fig. 9. Low-power micrograph showing variegated appearance. (Hematoxylin and eosin stain;
x 100.)
Fig. t0. High-power micrograph showing spindling and sarcomatoid appearance. (Hematoxytin and eosin stain; × 250.)
these variegated components, was malignant teratocarcinosarcoma. DISCUSSION
The term malignant teratocarcinosarcoma was derived by Heffner and Hyams I to describe a rare tumor of the sinonasal tract. Various other terms have been used to describe this tumor, including carcinosarcoma, malignant teratoma, and teratocarcinoma. The variegated histologic pattern was used to distinguish this tumor as a separate disease entity.
The histologic diagnosis is based on a combination of epithelial and connective tissue elements, such as fibroblasts, cartilage, bone, and, smooth muscle. If only a single mesenchymal component is present, the lesion is termed a carcinosarcoma. 2 Tissue heterogeneity and a variegated architectural pattern are characteristic of these teratoid lesions? The tumor is thought to originate from immature pleuripotential cells that have become sequestered in the sinonasal tract. Small samples, such as from biopsies, may not show the true histologic range of these tumors. This may account for the original biopsy
500
Otolaryngology Head and Neck Surgery March 1995
SHEMEN et al.
diagnosis in our case of malignant craniopharyngioma. Typical symptoms include nasal obstruction and epistaxis. Examination shows a polypoid mass. CT demonstrates opacification of one or several sinuses with bone destruction. These tumors are locally aggressive and may invade the orbit or cranium. In 6 (30%) of 20 cases cervical nodal metastasis was present or developed. Follow-up information was available for 17 cases: 8 had recurrence, 9 died with disease, and 1 was alive with disease. Five patients had no evidence of disease as long as 9 years later, and one died of an unrelated condition and was disease free at the time of death (30% 5-year survival). We reviewed six other cases in the literature with similar results. The recommended treatment for this condition was surgery and radiation therapy. Shanmugaratnam et al? reviewed three additional cases and also advocated postoperative radiation therapy. Devgan et al., 5 Christensen et al., 6 and Martinez et al. 7 each reviewed an additional case. Of the 20 cases reviewed by Heffner and Hyams in 1984,1 none originated primarily in the sphenoid sinus. Sites of origin have been described as the nasal cavity, maxillary sinus, ethmoid sinus, posterior choana, and orbit. Tumors were found in the sphenoid sinus, but these were thought to have been extensions from surrounding ethmoid disease. The CT and MRI scans, as well as the intraoperative findings, clearly show that the tumor presented here originated in the sphenoid sinus. A review of the literature shows no cases originating in the sphenoid sinus. The anatomy of the sphenoid sinus makes the diagnosis of disease arising in this particular sinus different from those in the other sinuses. The sphenoid begins to develop during the third year of life from an invagination of the sphenoethmoidal recess. It reaches adult size late in the second decade of life. As the sphenoid expands, it abuts adjacent neurologic and vascular structures. The optic nerves, carotid artery, and vidian nerves all lie in close proximity. In our case, the tumor exl~anded laterally to the right affecting the ophthalmic and maxillary branches of the trigeminal nerve and oculomotor
nerve. The tumor had eroded up to the optic chiasm. This, however, did not affect the patient's visual acuity. The return of normal sensation and motor function several weeks after surgery are consistent with a neuropraxic type of injury. The approach used for the surgical resection of this tumor offered maximum exposure of the sphenoid sinus. The complete rhinotomy and bilateral maxillectomy used in conjunction with a transsphenoid/transethmoid approach allowed for complete resection of the tumor with minimal morbidity. In addition, there was minimal disfigurement. \
SUMMARY
We have presented the first reported case of malignant teratocarcinosarcoma arising in the sphenoid sinus treated successfully with surgery and radiation therapy. The patient shows no signs of recurrence 5 years after surgery. The combined head and neck/neurosurgical approach using complete rhinotomy and medial maxillectomy with transsphenoid/transethmoid approach is advocated for sphenoid sinus tumors of this type. We would like to acknowledge Dede Silverstone for library assistance and Winston Harrison, MD, for the photomicrographs. REFERENCES
1. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma) of the nasal cavity and paranasal sinuses: a clinicopathologic study of 20 cases. Cancer 1984;53:2140-54. 2. Shindo ML, Stanley RB, Kiyabu MT. Carcinosarcoma of the nasal cavity and paranasal sinuses. Head Neck 1990;12:516-9. 3. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GSC, Sinniah R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413-9. 4. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GSC, Sinniah R. Terataid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413-9. 5. Devgan BK, Devgan M, Gross CW. Teratocarcinoma of the sphenoid sinus: review of the literature plus a new case report. OTOLARYNGOLI-IEADNECKSURG1978;86:689-95. 6. Christensen EC, Fu YS, Wilson LJ, Hoover LA. Teratoid carcinosarcoma of the nasal and paranasal cavities: a case report. Am J Rhinol 1992;6:169-72. 7. Martinez R, Lopez R, Parra R, Rodriguez B. Sinusal teratocarcinosarcoma. Acta Otorrinolaringol Esp 1991;42:363-7.