|Year : 2019 | Volume
| Issue : 1 | Page : 30-33
Chondrosarcoma at the sinonasal region
Santosh Kumar Swain1, Smrutipragnya Samal1, Mahesh Chandra Sahu2
1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to Be), Bhubaneswar, Odisha, India
2 Medical Research Laboratory, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to Be), Bhubaneswar, Odisha, India
|Date of Submission||04-Feb-2019|
|Date of Acceptance||10-Apr-2019|
|Date of Web Publication||20-Jun-2019|
Dr. Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to Be), K8, Kalinganagar, Bhubaneswar - 751 003, Odisha
Source of Support: None, Conflict of Interest: None
Chondrosarcoma is a rare and potentially lethal neoplasm and even rarer in the head-and-neck area. It is a very uncommon clinical condition at the sinonasal tract. The determination of this neoplasm depends on the histopathological consideration indicating harmful chondroid tissue multiplication. The prognostic elements for chondrosarcomas are grading of tumor and histological picture of tumor edges. The primary treatment of this tumor is radical surgery with wide safe margin. The other adjuvant treatments such as radiation and/or chemotherapy remain uncertain. The biopsy confirmed the diagnosis of myxoid chondrosarcoma. Here, we present a rare case of chondrosarcoma of a 48-year-old man presented with mass at the nasofacial region.
Keywords: Chondrosarcoma, maxillofacial area, sinonasal region, wide surgical excision
|How to cite this article:|
Swain SK, Samal S, Sahu MC. Chondrosarcoma at the sinonasal region. BLDE Univ J Health Sci 2019;4:30-3
Chondrosarcomas are rare malignancy, and its origin is from cartilaginous tissue or bone got from chondroid precursors. It represents around 10%–20% of all types of malignant bony tumors in the body whereas its incidence is rare in the head-and-neck region. In the head-and-neck area, it affects commonly the mandible, maxilla, nasal cavity, and paranasal sinuses. Rare location for chondrosarcomas in the head-and neck-region are the skull base and temporomandibular joint. The sinonasal tract has rarely been documented in the medical literature, and the maxillary sinus is the most common location to be affected; but, it rarely extends into the nasal cavity. The common clinical presentations of the patients with head-and-neck chondrosarcomas are localized swelling, mass formation, nasal obstruction, and sometimes pain. The prognosis of this disease is often variable and influenced by histological grading and site of origin.
Here, we are presenting a case report of chondrosarcoma showing a mass at the nasofacial area which originating at the maxilla and extending into the nasal area and ethmoidal sinuses.
| Case Report|| |
A 48-year-old man attended outpatient department of otorhinolaryngology with complaints of swelling at the left nasofacial region, left side nasal blockage, and occasional nasal bleeding for 1 year. He was a farmer by occupation, and on examination, there was diffuse swelling involving the left side of the face and lateral wall of the nose [Figure 1]. The consistency of the swelling is firm and ill-defined margin. The skin over the swelling was free but tense. There was no bulging in the palate during oral cavity examination. On anterior rhinoscopy, there was smooth swelling at the floor of the nose hence causing nasal obstruction, and blood-stained nasal discharge was present inside the nasal cavity. Fine-needle aspiration cytology showed inflammatory lesion. A diagnostic nasal endoscopy was done which showed firm bulging in the floor of the left nasal cavity. The left nasal cavity was narrowed due to pushing of lateral wall of the nose medially. The nasal cavity lining was reddish in look. The mucoid discharge from nasal cavity was sent for fungal culture in Sabouraud's medium and bacterial culture. Computed tomography (CT) scan showed mass involving the left maxilla, left ethmoid, and left side nasal cavity [Figure 2]. Orbit and cranium were within normal limits. The routine hematological tests were within normal limits. Under local anesthesia, a small piece of tissue was taken from the swelling inside the nasal cavity and sent for histopathological examination. The fungal and bacterial culture were negative.
|Figure 2: Computed tomography scan picture showing mass involving the left nasal cavity, maxillary, and ethmoid sinus|
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Microscopic picture shows myxoid chondrosarcoma with an abundant myxoid matrix containing round or slightly elongated cells with small hyperchromatic nuclei. The histopathological report was suggestive of myxoid chondrosarcoma [Figure 3]. He had undergone wide surgical excision by performing the left maxillectomy [Figure 4]. The mass is excised with healthy margin [Figure 5]. The follow-up at 1 year did not show any evidence of recurrence.
|Figure 3: Microphotography showing myxoid chondrosarcoma with an abundant myxoid matrix containing round or slightly elongated cells with small hyperchromatic nuclei (H and E, ×200)|
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| Discussion|| |
Chondrosarcoma is an uncommon malignant tumor at the head-and-neck territory speaks to under 0.1% of the tumors in this locale. It is a slow-growing neoplasm with high possibility of local recurrence. This tumor may arise from the ligament, delicate tissue, and bone. Common sites for origin of chondrosarcoma in the body are pelvic bone and long bones such as femur. The usual locales in the head-and neck-region for chondrosarcoma are mandible, nasal cavity, and paranasal sinuses. It is often seen in adults and rarely seen in the pediatric age group. The clinical behaviors of the chondrosarcoma are variable and in majority of the cases presented with low-grade malignant tumors as in our case. This tumor is relatively smaller in size at the maxillofacial regions and sinonasal tracts whereas it is typically large in the axial skeleton. CT scan often shows a well-defined mass with the presence of stippled calcified areas. Magnetic resonance imaging (MRI) report is quite variable. The noncalcified areas in MRI show isointense to hypointense signal intensity on T1-weighted images whereas hypointense on T2-weighted images. MRI with gadolinium contrast reveals inhomogeneous enhancement. The imaging findings often show variable calcification, lobulation, hypervascularity, and destruction of bone. The cut section of the neoplasm shows blue–gray fragments, with focal hemorrhagic spots. There is biphasic pattern of well-differentiated chondroid elements seen in the microscopic picture. There may be myxoid degeneration, and hemorrhagic necrosis may be seen. Tumor cells are seen around the vessels with a hemangiopericytoma-like pattern. In immunohistochemical study, the small round cells are mostly positive for CD99. The chondroid areas are often positive for S-100 proteins.
The diagnosis is often based on the histological study. Histologically, it is divided on the basis of microscopic appearance into customary, myxoid, clear cell, mesenchymal, and dedifferentiated chondrosarcoma. The traditional/classical and conventional chondrosarcomas are portrayed by a lobulated malignant cartilaginous tissue, mitosis, atomic pleomorphism, and binucleated cells. The histological classification for chondrosarcomas is graded from Grade I to III. The differential diagnosis includes small cell osteosarcoma, a small blue round cell neoplasm as Ewing's sarcoma or primitive neuroectodermal neoplasm, embryonal rhabdomyosarcoma, and lymphoma. The identification of undifferentiated mesenchymal cells and chondroid matrix is crucial for exact diagnosis of this neoplasm.
The objective of the treatment is to remove the tumor completely. Wide local or radical resection of the tumor is the favored treatment of chondrosarcoma. The surgical procedure of this tumor incorporates careful resection of the essential lesion with sufficient edge. If it is in the maxillary sinus, maxillectomy is regularly required. As the maxillary sinus anatomy is complex, total careful extraction of the chondrosarcoma with histological leeway of the edge is regularly needed. Chondrosarcoma is viewed as a radioresistant tumor. In some cases, the essential job of radiation treatment is for the treatment of unresectable lesion and if there should be an occurrence of deficiently resected tumors. Radiation treatment is frequently suggested in the event of postoperative adjuvant treatment for patients with high-grade lesion of chondrosarcomas. In few cases, preoperative and postoperative chemotherapy are useful. The rate of distant metastasis is uncommon and frequently found in further developed stage or recurrence cases. The distant metastasis is usually seen at the lungs, in spite of the fact that it might strike the vertebrae. In chondrosarcoma, the rate of distant and neighborhood metastasis is low and seen in roughly 5% of the cases. Metastasis happens fundamentally in the late phase of the tumor. The reason for death in patients of head-and-neck chondrosarcoma is local recurrence with tumor attacking the important or vital structures. The vital prognostic factors in head-and-neck chondrosarcoma are anatomical areas, histological staging, and wide excision. The most essential prognostic factor in chondrosarcoma is resectibility where complete excision is the absolute most factor for deciding the prognosis. This reality is upheld by the way that the most common reason for death in chondrosarcoma is recurrence of local site not because of metastasis. The careful treatment ought to be complete excision of local lesion even in instances of recurrence as the failure occurs at tumor site. The complete excision of the local area is of most extreme significance for effective result. Postoperative radiotherapy is useful in case of positive edges. The survival rate of the patients with head-and-neck chondrosarcoma is roughly 60% at 5 years and around half at 10 years.
| Conclusion|| |
The myxoid variety of chondrosarcoma at the nasofacial area is an extremely rare lesion and presented here because of rarity of this clinical entity. As it is a slow-growing tumor and resistant to radiation therapy, radical surgical excision is often considered as a good treatment option. There is a good chance of cure if it is diagnosed early. Postoperative follow-up is essential for monitoring the recurrence. Clinicians should be aware of this rare clinical entity and keep it in mind in case of differential diagnosis of maxillofacial mass so that delayed intervention is prevented.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Koch BB, Karnell LH, Hoffman HT, Apostolakis LW, Robinson RA, Zhen W, et al.
National cancer database report on chondrosarcoma of the head and neck. Head Neck 2000;22:408-25.
Munshi A, Atri SK, Pandey KC, Sharma MC. Dedifferentiated chondrosarcoma of the maxilla. J Cancer Res Ther 2007;3:53-5.
González-Pérez LM, Sánchez-Gallego F, Pérez-Ceballos JL, López-Vaquero D. Temporomandibular joint chondrosarcoma: Case report. J Craniomaxillofac Surg 2011;39:79-83.
Takahama A Jr., Alves Fde A, Prado FO, Lopes MA, Kowalski LP. Chondrosarcoma of the maxilla: Report of two cases with different behaviours. J Craniomaxillofac Surg 2012;40:e71-4.
Lee SY, Lim YC, Song MH, Seok JY, Lee WS, Choi EC, et al.
Chondrosarcoma of the head and neck. Yonsei Med J 2005;46:228-32.
Huang WC, Hu KY, Kuo KT, Tsai CC. Mesenchymal chondrosarcoma of the maxilla: Case report and clinicopathologic review. Tzu Chi Med J 2013;25:55-7.
Shapeero LG, Vanel D, Couanet D, Contesso G, Ackerman LV. Extraskeletal mesenchymal chondrosarcoma. Radiology 1993;186:819-26.
Tien N, Chaisuparat R, Fernandes R, Sarlani E, Papadimitriou JC, Ord RA, et al.
Mesenchymal chondrosarcoma of the maxilla: Case report and literature review. J Oral Maxillofac Surg 2007;65:1260-6.
Jörg S, August C, Stoll W, Alberty J. Myxoid chondrosarcoma of the maxilla in a pediatric patient. Eur Arch Otorhinolaryngol 2006;263:195-8.
Fayda M, Aksu G, Yaman Agaoglu F, Karadeniz A, Darendeliler E, Altun M, et al.
The role of surgery and radiotherapy in treatment of soft tissue sarcomas of the head and neck region: Review of 30 cases. J Craniomaxillofac Surg 2009;37:42-8.
Seidl RO, Gerein V, Vogel HJ. Mesenchymal chondrosarcoma of the facial skull. HNO 2001;49:744-9.
Liu ZQ, Tian Z, Ren ZH, Yang R, Cao W, Ji T. Chondrosarcoma in the head and neck region: A retrospective analysis of the clinicopathological features and treatment outcomes from a single institute database. Int J Oral Maxillofac Surg 2017;46:290.
Prado FO, Nishimoto IN, Perez DE, Kowalski LP, Lopes MA. Head and neck chondrosarcoma: Analysis of 16 cases. Br J Oral Maxillofac Surg 2009;47:555-7.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]