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Chondrosarcoma of the sphenoid sinus resected by an endoscopic approach
Chondrosarcoma of the Sphenoid Sinus Resected by an Endoscopic Approach Ricardo L. Carrau, MD, FACS,*† Barlas Aydogan, MD,‡ and Jennifer L. Hunt, MD§ Chondrosarcoma of the sinonasal tract is a rare neoplasm. Surgical excision is usually sufficient for low-grade lesions, whereas combined treatment is usually recommended for high-grade lesions. The oncologic outcome depends on a complete resection as well as the grade of the tumor. We present the case of a 35-year-old man presenting with an incidental finding of a chondrosarcoma of the posterior septum and rostrum of the sphenoid sinus. The tumor was completely resected using a transnasal endoscopic approach obtaining negative margins. Surgery is the mainstay treatment for low-grade chondrosarcomas. In selected patients, complete resection can be achieved using transnasal endoscopic approaches. (Am J Otolaryngol 2004;25:274-277. © 2004 Elsevier Inc. All rights reserved.)
Chondrosarcomas are rare, slow-growing tumors that usually arise from cartilaginous structures.1-7 The preferred treatment is surgical resection. Gross total removal with postoperative radiation is recommended for tumors involving vital structures. The reported rate of recurrence is high, varying from 40% to 60%1-7; however, the prognosis of patients with chondrosarcoma is influenced by the size of the tumor, its grade, and completeness of its resection. These factors correlate with the rate of metastasis, the local aggressiveness, and therefore, patients’ survival. We present a case of a rare chondrosarcoma of the nasal septum and rostrum of the sphenoid sinus resected via an endoscopic approach. CASE REPORT Our patient is a 35-year-old man who was diagnosed with a tumor arising at the posterior nasal septum found as an incidental find-
From the Departments of *Otolarynogology; and †Neurological Surgery, UMPC Center for Cranial Base Surgery, University of Pittsburgh, Pittsburgh, PA; ‡Department of Otolaryngology, University of Ankara, Ankara, Turkey; and §Department of Pathology, University of Pittsburgh, Pittsburgh, PA. Address correspondence to: Ricardo L. Carrau, MD, FACS, Department of Otolaryngology, University of Pittsburgh Medical Center, Eye and Ear Institute, Suite 500, 200 Lothrop Street, Pittsburgh, PA 15213. E-mail: [email protected]. © 2004 Elsevier Inc. All rights reserved. 0196-0709/$ - see front matter doi:10.1016/j.amjoto.2003.12.007 274
ing on magnetic resonance imaging performed as part of an infertility workup. At the time of diagnosis, the patient had no sinonasal symptoms. A computed tomography (CT) scan revealed a partially calcified mass with the epicenter at the junction of the nasal septum with the rostrum of the sphenoid sinus (Fig 1). The tumor involved and/or eroded the floor of the sphenoid sinus and the medial aspect of the right vidian canal (Fig 1B). Small focal regions of bone thinning, adjacent to the carotid canal, were also noted. The tumor did not extend intracranially or into the orbit; metastatic workup including a bone scan and a CT of the lungs and abdomen was negative for distant metastasis. Interestingly, a review of a prior CT scan performed at the time of a motor vehicle accident 3 years before our evaluation showed the presence of this tumor with minimal difference in size. The tumor was resected via an endonasal endoscopic approach. Bilateral sphenoidotomies were performed above the tumor. The posterior nasal arteries were clipped and transected bilaterally to facilitate the access to the vidian canals and the inferolateral quadrant of the sphenoid sinuses. The posterior nasal septum was transfixed, and the tumor was removed. The boundaries of the tumor were incised with sharp dissectors and/or a high-speed drill with a cutting burr. No packing was necessary, and the patient was discharged home the same day of surgery. Margins of resection were negative for tumor.
American Journal of Otolaryngology, Vol 25, No 4 (July-August), 2004: pp 274-277
CHONDROSARCOMA
Fig 1.
275
Coronal CT scan showing a chondrosarcoma arising at rostrum of the sphenoid sinus.
The patient tolerated the procedure well and was discharged home the same day of the surgery. The histological report revealed a chondrosarcoma that was graded between 1 (well differentiated) and 2 (moderately differentiated) on a scale of 1 to 3 (Fig 2). Subsequent CT scans performed at 4, 12, 24, and 36 months after the initial surgery, as well as multiple office endoscopies, revealed no evidence of disease (Fig 3). The patient continues to show no evidence of disease after 3 years of follow-up. DISCUSSION Chondrosarcomas can be divided into 2 main categories: primary chondrosarcomas are those that arise in normal cartilage and comprise the majority of chondrosarcomas. They are believed to arise from clearly differentiated cartilage cells; therefore, tissues that contain cartilage including the nasal septum, larynx, and those tissues that have ossified in cartilage such as the sphenoid bone are the
most common sites of origin for a chondrosarcoma. Secondary chondrosarcomas are those that arise in a preexistent chondroma and are extremely rare. In 1974, Fu and Perzin3 reviewed the medical literature and found 10 cases of chondrosarcoma involving the sinonasal tract. The average age of these patients was 44 years, which is a decade younger than chondrosarcomas arising elsewhere. The clinical and radiologic differential diagnosis includes chondroma, meningioma, osteomas, osteosarcomas, fibro-osseous lesions, and chordoma. The histological diagnosis of a chondrosarcoma may be difficult, and its differential diagnosis includes chondroma, osteogenic sarcoma, and salivary gland neoplasms. Chondromas may be difficult to differentiate from well-differentiated chondrosarcomas. Although chondromas are more prevalent in the sinonasal region, chondrosarcomas outnumber chondromas. Adequate biopsy specimens are therefore necessary to avoid sampling errors. Plumpness to the cells
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Fig 2. Background image (hematoxylin and eosin–stained section, 10ⴛ original magnification). This is a lowpower photomicrograph of the chondrosarcoma (CS) located just below the sinonasal mucosa (arrows). The tumor shows a sheet of cartilaginous matrix containing hypercellular chondrocytes with variable but mild atypia. Inset image (hematoxylin and eosin–stained section, 40ⴛ original magnification). This high-power photomicrograph shows the hypercellular nature of the chondrocytes, as well as cytologic atypia. Multiple binucleated chondrocytes were also present (arrowhead).
Fig 3. Postoperative coronal CT scan showing a complete resection.
and multinucleated cells with irregular hyperchromatic nuclei is useful to establish the diagnosis. Although there may be areas of calcification or ossification related to the tumor invasion of bone, there should be no osteoid or bone formation within the tumor. Lesions greater than 2 cm are considered chondrosarcomas despite a benign-appearing histology. Chondrosarcomas are graded on a 1 to 3 scale corresponding to a histological classification from a well-differentiated to undifferentiated tumor, based on the rate of mitoses, cellularity, and nuclear size. The absence of mitoses, however, does not eliminate the diagnosis of a malignancy. Their clinical course reflects their insidious growth and most often is asymptomatic at the time of diagnosis even
CHONDROSARCOMA
when they frequently displace adjacent structures before invading them. Surgical excision with wide surgical margins is the mainstay treatment. Chondrosarcoma is considered to be radioresistant, although complete response to radiotherapy has been reported sporadically in the literature.8 Radiation therapy is recommended for those patients with positive margins, those with chondrosarcoma extending to vital structures that impeded wide resection of the tumor, and those with recurrent or high-grade tumors. Regional and distant metastasis are rare. Distant metastasis is reported in less than 10% of these patients.1-7 In 1984, Nishizawa et al4 reviewed all the English literature and found 14 chondrosarcomas of the nasal septum. They combined the data provided by this review with the data of 7 other cases reported in the Japanese literature. At the time of their report, 5 patients (24%) had been lost to follow-up and 4 patients (20%) were alive with disease or dead of disease. Surgical extirpation can be completed by using a variety of approaches such as lateral rhinotomy with or without medial maxillectomy, the transpalatal approach, Le Fort I osteotomy, and transnasal endoscopic and microscopic approaches. Visualization of the tumor, protection of neurovascular structures, hemostasis, and avoidance of cosmetic deformities are important considerations when choosing the surgical approach. The choice of the approach is also influenced by the needs and age of the patient and the surgeon’s experience and training. The endoscopic approach avoids facial and oral incisions and produces the least morbid-
277
ity. Its use is limited by the access provided by the nasal cavity and paranasal sinuses, the vascularity, size, and extension of the tumor, and the availability of the special instrumentation. CONCLUSION This case report shows the feasibility of using endoscopic techniques to aid in the resection of selected malignant tumors of the nose. Although we do not advocate the use of endoscopic approaches for all malignant tumors, tumors that are limited in size and fully visualized by endoscopic techniques are amenable to endoscopic resection. The visualization provided by the endoscopes facilitates the outpatient monitoring of the patient. REFERENCES 1. Barnes L: Chondroma and chondrosarcoma of the craniofacial bones. Surg Pathol Head Neck 2:960-962, 1985 2. Drucker C, Sanders A, Bowes A, et al. Chondrosarcoma of the sphenoethmoid complex. Ear Nose Throat J 69:630-632, 1990 3. Fu Y, Perzin K: Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A clinicopathologic study. Cancer 34:453-463, 1974 4. Nishizawa S, Fukaya T, Inouye K: Chondrosarcoma of the nasal septum: A report of an uncommon lesion. Laryngoscope 94:550-553, 1984 5. Ishida M, Ashida K, Matsunaga T, et al: Chondrosarcoma of the ethmoidal and sphenoidal sinuses: A case of chondrosarcoma arising from the postparanasal sinuses. ORL 48:174-179, 1986 6. Gallagher T, Strome M: Chondrosarcomas of the facial region. Laryngoscope 82:978-984, 1972 7. Coates H, Pearson B, Devine K, et al. Chondrosarcoma of the nasal cavity, paranasal sinuses and nasopharynx. Am Acad Ophthalmol Otol 84:919-926, 1977 8. Lott S, Bordley J. A radiosensitive chondrosarcoma of the sphenoid sinus and base of the skull: Report of a case. Laryngoscope 82:57-60, 1972
Chondrosarcomas are rare, slow-growing tumors that usually arise from cartilaginous structures.1-7 The preferred treatment is surgical resection. Gross total removal with postoperative radiation is recommended for tumors involving vital structures. The reported rate of recurrence is high, varying from 40% to 60%1-7; however, the prognosis of patients with chondrosarcoma is influenced by the size of the tumor, its grade, and completeness of its resection. These factors correlate with the rate of metastasis, the local aggressiveness, and therefore, patients’ survival. We present a case of a rare chondrosarcoma of the nasal septum and rostrum of the sphenoid sinus resected via an endoscopic approach. CASE REPORT Our patient is a 35-year-old man who was diagnosed with a tumor arising at the posterior nasal septum found as an incidental find-
From the Departments of *Otolarynogology; and †Neurological Surgery, UMPC Center for Cranial Base Surgery, University of Pittsburgh, Pittsburgh, PA; ‡Department of Otolaryngology, University of Ankara, Ankara, Turkey; and §Department of Pathology, University of Pittsburgh, Pittsburgh, PA. Address correspondence to: Ricardo L. Carrau, MD, FACS, Department of Otolaryngology, University of Pittsburgh Medical Center, Eye and Ear Institute, Suite 500, 200 Lothrop Street, Pittsburgh, PA 15213. E-mail: [email protected]. © 2004 Elsevier Inc. All rights reserved. 0196-0709/$ - see front matter doi:10.1016/j.amjoto.2003.12.007 274
ing on magnetic resonance imaging performed as part of an infertility workup. At the time of diagnosis, the patient had no sinonasal symptoms. A computed tomography (CT) scan revealed a partially calcified mass with the epicenter at the junction of the nasal septum with the rostrum of the sphenoid sinus (Fig 1). The tumor involved and/or eroded the floor of the sphenoid sinus and the medial aspect of the right vidian canal (Fig 1B). Small focal regions of bone thinning, adjacent to the carotid canal, were also noted. The tumor did not extend intracranially or into the orbit; metastatic workup including a bone scan and a CT of the lungs and abdomen was negative for distant metastasis. Interestingly, a review of a prior CT scan performed at the time of a motor vehicle accident 3 years before our evaluation showed the presence of this tumor with minimal difference in size. The tumor was resected via an endonasal endoscopic approach. Bilateral sphenoidotomies were performed above the tumor. The posterior nasal arteries were clipped and transected bilaterally to facilitate the access to the vidian canals and the inferolateral quadrant of the sphenoid sinuses. The posterior nasal septum was transfixed, and the tumor was removed. The boundaries of the tumor were incised with sharp dissectors and/or a high-speed drill with a cutting burr. No packing was necessary, and the patient was discharged home the same day of surgery. Margins of resection were negative for tumor.
American Journal of Otolaryngology, Vol 25, No 4 (July-August), 2004: pp 274-277
CHONDROSARCOMA
Fig 1.
275
Coronal CT scan showing a chondrosarcoma arising at rostrum of the sphenoid sinus.
The patient tolerated the procedure well and was discharged home the same day of the surgery. The histological report revealed a chondrosarcoma that was graded between 1 (well differentiated) and 2 (moderately differentiated) on a scale of 1 to 3 (Fig 2). Subsequent CT scans performed at 4, 12, 24, and 36 months after the initial surgery, as well as multiple office endoscopies, revealed no evidence of disease (Fig 3). The patient continues to show no evidence of disease after 3 years of follow-up. DISCUSSION Chondrosarcomas can be divided into 2 main categories: primary chondrosarcomas are those that arise in normal cartilage and comprise the majority of chondrosarcomas. They are believed to arise from clearly differentiated cartilage cells; therefore, tissues that contain cartilage including the nasal septum, larynx, and those tissues that have ossified in cartilage such as the sphenoid bone are the
most common sites of origin for a chondrosarcoma. Secondary chondrosarcomas are those that arise in a preexistent chondroma and are extremely rare. In 1974, Fu and Perzin3 reviewed the medical literature and found 10 cases of chondrosarcoma involving the sinonasal tract. The average age of these patients was 44 years, which is a decade younger than chondrosarcomas arising elsewhere. The clinical and radiologic differential diagnosis includes chondroma, meningioma, osteomas, osteosarcomas, fibro-osseous lesions, and chordoma. The histological diagnosis of a chondrosarcoma may be difficult, and its differential diagnosis includes chondroma, osteogenic sarcoma, and salivary gland neoplasms. Chondromas may be difficult to differentiate from well-differentiated chondrosarcomas. Although chondromas are more prevalent in the sinonasal region, chondrosarcomas outnumber chondromas. Adequate biopsy specimens are therefore necessary to avoid sampling errors. Plumpness to the cells
276
CARRAU, AYDOGAN, AND HUNT
Fig 2. Background image (hematoxylin and eosin–stained section, 10ⴛ original magnification). This is a lowpower photomicrograph of the chondrosarcoma (CS) located just below the sinonasal mucosa (arrows). The tumor shows a sheet of cartilaginous matrix containing hypercellular chondrocytes with variable but mild atypia. Inset image (hematoxylin and eosin–stained section, 40ⴛ original magnification). This high-power photomicrograph shows the hypercellular nature of the chondrocytes, as well as cytologic atypia. Multiple binucleated chondrocytes were also present (arrowhead).
Fig 3. Postoperative coronal CT scan showing a complete resection.
and multinucleated cells with irregular hyperchromatic nuclei is useful to establish the diagnosis. Although there may be areas of calcification or ossification related to the tumor invasion of bone, there should be no osteoid or bone formation within the tumor. Lesions greater than 2 cm are considered chondrosarcomas despite a benign-appearing histology. Chondrosarcomas are graded on a 1 to 3 scale corresponding to a histological classification from a well-differentiated to undifferentiated tumor, based on the rate of mitoses, cellularity, and nuclear size. The absence of mitoses, however, does not eliminate the diagnosis of a malignancy. Their clinical course reflects their insidious growth and most often is asymptomatic at the time of diagnosis even
CHONDROSARCOMA
when they frequently displace adjacent structures before invading them. Surgical excision with wide surgical margins is the mainstay treatment. Chondrosarcoma is considered to be radioresistant, although complete response to radiotherapy has been reported sporadically in the literature.8 Radiation therapy is recommended for those patients with positive margins, those with chondrosarcoma extending to vital structures that impeded wide resection of the tumor, and those with recurrent or high-grade tumors. Regional and distant metastasis are rare. Distant metastasis is reported in less than 10% of these patients.1-7 In 1984, Nishizawa et al4 reviewed all the English literature and found 14 chondrosarcomas of the nasal septum. They combined the data provided by this review with the data of 7 other cases reported in the Japanese literature. At the time of their report, 5 patients (24%) had been lost to follow-up and 4 patients (20%) were alive with disease or dead of disease. Surgical extirpation can be completed by using a variety of approaches such as lateral rhinotomy with or without medial maxillectomy, the transpalatal approach, Le Fort I osteotomy, and transnasal endoscopic and microscopic approaches. Visualization of the tumor, protection of neurovascular structures, hemostasis, and avoidance of cosmetic deformities are important considerations when choosing the surgical approach. The choice of the approach is also influenced by the needs and age of the patient and the surgeon’s experience and training. The endoscopic approach avoids facial and oral incisions and produces the least morbid-
277
ity. Its use is limited by the access provided by the nasal cavity and paranasal sinuses, the vascularity, size, and extension of the tumor, and the availability of the special instrumentation. CONCLUSION This case report shows the feasibility of using endoscopic techniques to aid in the resection of selected malignant tumors of the nose. Although we do not advocate the use of endoscopic approaches for all malignant tumors, tumors that are limited in size and fully visualized by endoscopic techniques are amenable to endoscopic resection. The visualization provided by the endoscopes facilitates the outpatient monitoring of the patient. REFERENCES 1. Barnes L: Chondroma and chondrosarcoma of the craniofacial bones. Surg Pathol Head Neck 2:960-962, 1985 2. Drucker C, Sanders A, Bowes A, et al. Chondrosarcoma of the sphenoethmoid complex. Ear Nose Throat J 69:630-632, 1990 3. Fu Y, Perzin K: Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A clinicopathologic study. Cancer 34:453-463, 1974 4. Nishizawa S, Fukaya T, Inouye K: Chondrosarcoma of the nasal septum: A report of an uncommon lesion. Laryngoscope 94:550-553, 1984 5. Ishida M, Ashida K, Matsunaga T, et al: Chondrosarcoma of the ethmoidal and sphenoidal sinuses: A case of chondrosarcoma arising from the postparanasal sinuses. ORL 48:174-179, 1986 6. Gallagher T, Strome M: Chondrosarcomas of the facial region. Laryngoscope 82:978-984, 1972 7. Coates H, Pearson B, Devine K, et al. Chondrosarcoma of the nasal cavity, paranasal sinuses and nasopharynx. Am Acad Ophthalmol Otol 84:919-926, 1977 8. Lott S, Bordley J. A radiosensitive chondrosarcoma of the sphenoid sinus and base of the skull: Report of a case. Laryngoscope 82:57-60, 1972