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Year : 2020  |  Volume : 16  |  Issue : 3  |  Page : 653-656

Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma

1 Department of Radiation Oncology, Eulji University Hospital, Daejeon, Korea
2 Department of Pathology, Eulji University Hospital, Daejeon, Korea

Date of Submission15-Apr-2019
Date of Decision09-Jun-2019
Date of Acceptance20-Oct-2019
Date of Web Publication18-Jul-2020

Correspondence Address:
Mi-Jo Lee
Department of Radiation Oncology, Eulji University Hospital, 95, Dunsanseo-ro, Seogu, Daejeon 35233
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_254_19

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 > Abstract 

Nasopharyngeal sarcomatoid carcinoma (SaCa) is extremely rare, and concurrent chemoradiation is the standard treatment for squamous cell-based nasopharyngeal cancer (NPC). This case report gives the first explanation of a nasopharyngeal SaCa patient treated with volumetric modulated arc therapy (VMAT) with simultaneous integrated boost (SIB), which is an excellent treatment modality that leads to complete response for locally advanced NPC. A 70-year-old male presented with nasal obstruction, epistaxis, and right neck node enlargements. Examination revealed an extensive tumor of nasopharyngeal tumor extending into the nasal cavity and right parapharyngeal space with bilateral lymphadenopathy on positron emission tomography (PET)–computed tomography images of focal hypermetabolic bone lesion in C4 body (stage T3N2M1). An excisional biopsy of nasopharyngeal wall mass showed a SaCa. He received concurrent chemoradiation which was VMAT and systemic chemotherapy (cisplatin 60 mg). A dose of 70 Gy was delivered to the planning target volume (PTV70) (gross tumor volume plus margin 3–5 mm) and PTV59.4(a wider margin around high-risk clinical target volume, including the clivus and neck nodes) all given in 33 fractions. Radiological examination such as magnetic resonance imaging (MRI) and PET images at the completion of external beam therapy revealed questionable residual disease. Follow-up MRI scans 4 weeks after radiotherapy revealed a complete tumor response. VMAT with SIB can be an effective treatment option for SaCa of the advanced nasopharynx.

Keywords: Nasopharyngeal cancer, radiation therapy, sarcomatoid carcinoma, simultaneous integrated boost, volumetric arc therapy

How to cite this article:
Lee MJ, Son HJ. Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma. J Can Res Ther 2020;16:653-6

How to cite this URL:
Lee MJ, Son HJ. Complete response to radiation therapy for nasopharyngeal sarcomatoid carcinoma. J Can Res Ther [serial online] 2020 [cited 2020 Aug 7];16:653-6. Available from: http://www.cancerjournal.net/text.asp?2020/16/3/653/289968

 > Introduction Top

Sarcomatoid carcinoma (SaCa) (spindle cell) of the nasopharynx is a malignant tumor accomplished biphasic feature of malignant epithelial and connective tissues. SaCa is a very rare tumor, Western report not existed, just a few studies reported accounting for <0.3% of upper digestive tract SaCas in Taiwan.[1] The most common sites of SaCa are the larynx, oral, and sinus.[2] SaCa is an aggressive malignant head-and-neck tumor; it has worse 5-year overall survival than squamous cell nasopharyngeal carcinoma (NPC) (38% vs. 72%).[1],[3] The treatment of SaCa of the nasopharynx is not standardized because of its rarity; concurrent chemoradiation reported curable treatment option as like squamous cell origin nasopharyngeal carcinoma. In 1980 area, three-dimensional conformal radiation therapy has been used in radiotherapy (RT) for decades, but in recent years, volumetric arc therapy (VMAT) has become the standard of radiation therapy for head-and-neck malignancies. VMAT with simultaneous integrated boost (SIB) is one of the most delicate forms of RT. VMAT with SIB uses precision and accuracy dose painting to target tumor volumes such that normal organs receive a low dose per fraction.

This case study presents a patient with SaCa of the nasopharynx who showed a short-term complete tumor response to VMAT with SIB and without severe toxicity in time treatment.

 > Case Report Top

A 70-year-old patient visited our facility with a chief complaint of nasal obstruction and neck mass for several months. Obstruction symptom was associated with ear fullness and epistaxis. There was no hoarseness, dysphagia, or anorexia. There was no family history of malignancy or previously radiation history. He quit smoking 10 years ago and had a smoking capacity of 40.

On laryngoscopy examination, there was a mass-occupying posterior right nasal cavity. His vocal cords appeared equal and fully mobile, and there was no obvious mass. Both the external auditory canal and tympanic membranes appeared normal. Both neck nodes were palpable, especially the right Level II–III lymph node sized 1.5 cm.

Histopathology indicated that the mass was consistent with SaCa. The carcinoma component of the malignant cells was positive for Epstein–Barr virus by in situ hybridization, indicating a nasopharyngeal origin. The sarcomatous elements were positive for cytokeratin (Pan-CK, CK7, CK20, CK19, HM-CK, and CK5/6), EMA, and CD5 and negative for LCA, CD3, CD20, CD30, and NK cell markers (CD56, TIA-1, and granzyme B), which excluded a diagnosis of lymphoma. [Figure 1] shows the pathological diagnosis.
Figure 1: (a) H and E, ×200, (b) Pan-CK, (c) Vimentin, (d) CD5, (e) Epstein–Barr virus in situ hybridization

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Magnetic resonance imaging (MRI) of the nasopharynx showed a relatively homogeneous, T2-intermediate, T1-isosignal-enhancing mass or mass-like lesion from the posterior wall mass extension of the nasopharynx to the right nasal cavity and right ethmoid sinus with clivus invasion. Multiple enlarged lymph nodes with necrosis were observed in the right retropharyngeal area and both Level II and III cervical lymph node chains bilaterally (largest measuring 1.5 cm × 0.9 cm). Positron emission tomography–computed tomography (PET–CT) indicated a hypermetabolic bone lesion at the C4 spinal and skull bases [Figure 2].
Figure 2: (a) Magnetic resonance imaging and (b) positron emission tomography–computed tomography before treatment. (c and d) Near complete response 4 weeks after treatment (c: magnetic resonance imaging, d: positron emission tomography–computed tomography)

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The patient underwent systemic chemotherapy (cisplatin) and referred to the radiation oncology department, where he underwent 70 Gy of radiation therapy. Normal organs prescription dose is demonstrated in [Table 1].
Table 1: Normal organ prescription (late effect and QUANTEC in Perez and Brady's Principle and practice of radiation oncology 7th edition p360-1)

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During the simulation, the patient was immobilized in the supine position with his arms above his head using aqua plaster. CT was conducted using a 16-slice CT scanner (Somatom Sensation 16; Siemens Healthcare, Germany).

Treatment planning was performed with Monaco 5.0 (Elekta AB, Stockholm, Sweden) using the X-ray voxel Monte Carlo dose algorithm for a Synergy linear accelerator (Elekta, England). The entire isodose curve is demonstrated in [Figure 3].
Figure 3: Volumetric modulated arc therapy plan with the planning target volume. Dose distribution with isodose lines, from 100% to 30%

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MRI and PET–CT 1 month after the completion of RT showed that the nasopharyngeal mass had almost disappeared. In addition, there were no palpable neck nodes. Patients are dead after 2-month complete radiation treatment. Cause death is the dissemination of bony metastasis.

 > Discussion Top

This male patient presented with no known additional risk factors.[2],[6],[7] He denied any history of alcohol consumption or a previous history of radiation exposure. He presented to us only with a 3-week history of nasal obstruction/bleeding, and he was already noted to have posterior nasal mass sensation with neck metastasis. VMAT with SIB uses high-precision dose painting to target tumors such that normal organs receive a low dose per fraction. This case report presents a patient with SaCa of the nasopharynx who showed a complete tumor response to VMAT with SIB, and there was no in-field recurrence or severe toxicity except mild respiration system side effect.

NPC is the most common tumor of the nasopharynx. SaCas include synovium spindle cell carcinoma, pseudosarcoma, carcinosarcoma, and unusual variants of squamous carcinoma.[4] SaCa has previously been reported in the upper aerodigestive tract, prostate, and small intestine.[1],[2],[4],[5]

Although the head-and-neck area is a common site for SaCa development, very little has been written on this disease.[1],[2] SaCa is a male-predominant disease, with older age, alcohol consumption, tobacco use, and previous RT as additional risk factors.[2],[6],[7] The mortality rate is higher than that in squamous cell carcinoma, with better outcomes associated with early-stage and extraoral tumors.[2],[8] The high mortality rate of SaCa in the head-and-neck area is believed to be due to lower resistance to tumor spreading along tissue planes and the extensive lymph drainage system, with resultant lymphatic and distant metastases.[6],[9],[10]

In a series of 78 Taiwan cases collected from an extensive head-and-neck cancer database from 1978 to 2008, only three were nasopharyngeal SaCa.[1] These patients were treated with RT, but that study included no description about RT administration. Among the carcinosarcoma of the head and neck, laryngeal sarcomatoid tumors seem to have a better prognosis compared to extralaryngeal tumors. They also reported a high tendency for local recurrence, distant metastasis, and poor outcome. In patients who received concurrent chemoradiotherapy, only 36% (four of 11) underwent complete remission; the other seven patients died within 6 months from the uncontrollable disease. The current concept for the treatment of SaCa is RT when the tumor is in the nasal cavity, nasopharynx, or hypopharynx, where RT remains the primary treatment modality.

SaCa of the nasopharynx, although rare, is not to be taken lightly by health professionals. It is known to be very aggressive and has a poor prognosis. Therefore, early diagnosis and prompt treatment are essential to improve the outcome and survival rates of these patients. More research regarding the treatment modalities for nasopharyngeal SaCa is still needed.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Chang NJ, Kao DS, Lee LY, Chang JW, Hou MM, Lam WL, et al. Sarcomatoid carcinoma in head and neck: A review of 30 years of experience – Clinical outcomes and reconstructive results. Ann Plast Surg 2013;71 Suppl 1:S1-7.  Back to cited text no. 1
Thompson LD, Wieneke JA, Miettinen M, et al. Spindle cell (sarcomatoid) carcinomas of the larynx: A clinicopathologic study of 187 cases. Am J Surg Pathol 2002;26:153Y170.  Back to cited text no. 2
Kamran SC, Riaz N, Lee N. Nasopharyngeal carcinoma. Surg Oncol Clin N Am 2015;24:547-61.  Back to cited text no. 3
Delahunt B, Eble JN, Nacey JN, Grebe SK. Sarcomatoid carcinoma of the prostate: Progression from adenocarcinoma is associated with p53 over-expression. Anticancer Res 1999;19:4279-83.  Back to cited text no. 4
Fukuda T, Kamishima T, Ohnishi Y, Suzuki T. Sarcomatoid carcinoma of the small intestine: Histologic, immunohistochemical and ultrastructural features of three cases and its differential diagnosis. Pathol Int 1996;46:682-8.  Back to cited text no. 5
Leventon GS, Evans HL. Sarcomatoid squamous cell carcinoma of the mucous membranes of the head and neck: A clinicopathologic study of 20 cases. Cancer 1981;48:994Y1003.  Back to cited text no. 6
Ellis GL, Corio RL. Spindle cell carcinoma of the oral cavity. A clinicopathologic assessment of fifty-nine cases. Oral Surg Oral Med Oral Pathol 1980;50:523Y533.  Back to cited text no. 7
Takata T, Ito H, Ogawa I, Miyauchi M, Ijuhin N, Nikai H, et al. Spindle cell squamous carcinoma of the oral region. An immunohistochemical and ultrastructural study on the histogenesis and differential diagnosis with a clinicopathological analysis of six cases. Virchows Arch A Pathol Anat Histopathol 1991;419:177-82.  Back to cited text no. 8
Onishi H, Kuriyama K, Komiyama T, Yamaguchi M, Tanaka S, Marino K, et al. T1N0 laryngeal sarcomatoid carcinoma that showed rapid systemic metastases after radical radiotherapy: A case report and review of literature. Am J Otolaryngol 2005;26:400-2.  Back to cited text no. 9
Hyams VJ. Spindle cell carcinoma of the larynx. Can J Otolaryngol 1975;4:307-13.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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