Journal of Laryngology and Voice

CASE REPORT
Year
: 2020  |  Volume : 10  |  Issue : 1  |  Page : 10--14

Case report: Rare cases of primary retropharyngeal synovial sarcoma and rhabdomyosarcoma


Suresh Singh Naruka1, Sandeep S Sindhu1, Venus Rawat1, Seema Singhal2, VA Nasyrov3,  
1 Department of Otorhinolaryngology, Head and Neck Surgery, Indraprastha Apollo Hospital, Delhi, India
2 Department of Pathology, Indraprastha Apollo Hospital, Delhi, India
3 Department of Otorhinolaryngology, Head and Neck Surgery, Kyrgyz Medical Academy, Bishkek, Kyrgyzstan

Correspondence Address:
Venus Rawat
J-402, KDP Grand Savanna, Raj Nagar Extension, Ghaziabad - 201 017, Uttar Pradesh
India

Abstract

Synovial sarcoma is the third most common histologic type soft tissue sarcoma of extremities. However, primary retropharyngeal synovial sarcoma is extremely rare. Rhabdomyosarcoma occurs frequently in children and adolescents. In contrast, it is rare in adults and is not well characterized clinically and pathologically. We present case report of two cases of retropharyngeal sarcomas, one synovial sarcoma in a young male and another rhabdomyosarcoma in a young female patient. Diagnosis is based on history, clinical assessment and CT imaging findings. Management comprises of surgical excision followed by chemotherapy. Though rare, these retropharyngeal tumours should always be kept in mind which could otherwise be missed easily even by a skilful surgeon. With early diagnosis and management of retropharyngeal tumours, the prognosis can be improved substantially.



How to cite this article:
Naruka SS, Sindhu SS, Rawat V, Singhal S, Nasyrov V A. Case report: Rare cases of primary retropharyngeal synovial sarcoma and rhabdomyosarcoma.J Laryngol Voice 2020;10:10-14


How to cite this URL:
Naruka SS, Sindhu SS, Rawat V, Singhal S, Nasyrov V A. Case report: Rare cases of primary retropharyngeal synovial sarcoma and rhabdomyosarcoma. J Laryngol Voice [serial online] 2020 [cited 2020 Nov 24 ];10:10-14
Available from: https://www.laryngologyandvoice.org/text.asp?2020/10/1/10/299957


Full Text



 Introduction



The retropharyngeal space is a potential space of the head and neck bounded by buccopharyngeal fascia anteriorly and the alar fascia posteriorly. It contains retropharyngeal lymph nodes. Synovial sarcoma is a mesenchymal spindle-cell tumor that displays variable epithelial differentiation, including glandular formation. Synovial sarcoma accounts for 5–10% of soft-tissue sarcomas.[1] Spindle cell sarcoma is an extremely rare entity to present in the retropharyngeal spaces. Rhabdomyosarcoma (RMS) is the most common pediatric soft-tissue sarcoma, constituting 3%–5% of all malignancies in childhood.[2] The diagnosis and management of retropharyngeal synovial sarcoma and RMS are highly challenging.

 Case Reports



Case report 1

An 18-year-old male patient presented to the hospital with complaints of swelling in the left side of the neck, difficulty in swallowing for both solids and liquids, difficulty in breathing, hoarseness, and occasional episodes of hematemesis. The patient also gave a history of weight loss. On examination, there was diffuse swelling anteriorly over the left side of the neck, firm in consistency, smooth surface, nontender, nonpulsatile, irreducible, and the skin over the swelling was pinchable. On the oropharyngeal examination, the retropharyngeal bulge was visualized on the left side. No neck nodes palpated. Flexible fiberoptic laryngoscopy was done which was suggestive of the bulge in the retropharyngeal region along with large soft-tissue mass in the hypopharynx and left side of supraglottis. Fine-needle aspiration cytology (FNAC) of the neck swelling was done, which was suggestive of nodular goiter. Ultrasound neck showed a nodular goitre. The patient was advised contrast-enhanced high-resolution computed tomography (HRCT) of the neck [Figure 1].{Figure 1}

HRCT was suggestive of the homogeneous hypodense lesion in the hypopharynx, predominantly on the left side extending from C3 to C6 level, measuring 5.2 (CC) × 4.8 (AP) cm. It was showing heterogeneous contrast enhancement. It involved the left pyriform fossa with narrowing in the supraglottic airway. Intraoperatively, the large hematoma was seen in the hypopharynx, which was removed. Incision was given over retropharyngeal bulge, and the tumor was excised and sent for histopathology. The patient recovered well. Biopsy [Figure 2] was suggestive of monomorphic cells forming lobules, fascicles, and cords. The cells had indistinct cytoplasm, spindle shaped nuclei. Focal whorling was seen. Focal mast cells and atypical mitoses were present. No necrosis was seen. Histomorphology was suggestive of malignant spindle cell tumour.{Figure 2}

Immunohistochemistry [Figure 3] for EMA, vimentin, CD99, Bcl2 was diffusely positive in tumor cells and CK AE1/AE3 was negative suggestive of synovial sarcoma (monophasic). The patient was advised chemotherapy. The patient is on follow-up and is doing well.{Figure 3}

Case report 2

A 22-year-old female patient came with the complaint of difficulty in swallowing for both solids and liquids for 4 months. It was insidious in onset, gradually progressive, and not relieved after taking medications. The patient gave a history of difficulty in breathing, especially in the supine position. She also noticed some changes in voice, though not very significant. On oropharyngeal examination, swelling was seen in the retropharyngeal region with a smooth surface [Figure 4].{Figure 4}

On neck examination, bilateral level Ib, II, III, and IV nodes were palpable. Contrast-enhanced computed tomography (CT) of the neck [Figure 5] was done which showed a well-defined, heterogeneously enhancing soft-tissue density lesion in the retropharyngeal space more toward right side extending from the level of C2 to C4 vertebral body and measuring 23.4 mm × 30.9 mm × 36 mm. The lesion was anteriorly displacing the epiglottis, and there was effacement of the right vallecula.{Figure 5}

FNAC showed scanty material, some polymorphs, lymphocytes, and few ill-defined collections of histiocytes were seen. Features were suggestive of the inflammatory lesion, but no definitive opinion could be made due to scanty aspirate. The patient was planned for the surgery, and tumor was excised with wide margins and sent for histopathology. Biopsy [Figure 6].showed tumor comprising a mixture of small, undifferentiated, hyperchromatic round, and spindle-shaped cells with ovoid pleomorphic nuclei, arranged in sheets and ill-defined fascicles. Interspersed were round-to-polygonal cells with markedly atypical nuclei, several multinucleated giant cells, and few bizarre cells. Focal anaplasia was noted. Suggestive of the malignant mesenchymal tumor (FNCLCC Grade 3) of the retropharyngeal region. Immunohistochemistry [Figure 7] was positive for vimentin and desmin, focally positive for CD 68 and negative for S-100. Features are indicative of Embryonal RMS (FNCLCC Grade 3) of the retropharyngeal region. Postoperatively, the patient underwent magnetic resonance imaging (MRI) of the neck [Figure 8], which was suggestive of soft-tissue thickening in the right side of the posterior pharyngeal wall at C2–C3 vertebral level measuring 1.2 cm × 1.8 cm × 2.1 cm closely abutting right posterior aspect of the epiglottis and right aryepiglottic fold.{Figure 6}{Figure 7}{Figure 8}

The lesion showed mild contrast enhancement in the anterior aspect suggestive of the residual or recurrent lesion. Positron emission tomography scan [Figure 9] was also done, which revealed metabolically active ill-defined asymmetrical soft-tissue thickening involving the right retropharyngeal region extending across C2-C3 vertebral bodies and 1.2 cm × 1.1 cm × 1.3 cm likely to be local residual disease.{Figure 9}

Mildly metabolically active few bilateral cervical lymph nodes were seen, probably benign in nature. Later, the patient was advised chemotherapy (vincristine, dactinomycin, and cyclophosphamide). The patient is on follow-up.

 Discussion



Synovial sarcoma is a mesenchymal spindle cell tumor that accounts for 5–10% of soft-tissue sarcomas.[1] Its annual incidence is 2.57/100,000.[3] They are reported from birth to 89 years of age, but occur mainly between the age of 15 and 40 years[4] and are more common in males. In case report 1, the tumor was seen in the 18-years-old male patient, which in accordance with the literature. Most of these tumors, around 80%, arise in the deep soft tissue of the extremities. Approximately, 5% arise in the head-and-neck region; however, any site can be affected.[5],[6] In our case report 1, the tumor was present in the retropharyngeal region, which is extremely rare.

The nodal pattern of retropharyngeal space is identified on CT or MRI images. Metastatic disease in the retropharyngeal space can result in nodal necrosis.[7] Synovial sarcoma is treated surgically with or without adjuvant radiation. Up to 50% of synovial sarcomas recur, usually within 2 years.[8] Some (40%) metastasize, commonly to the lungs and bones, and also to the regional lymph nodes. The prognosis does not differ between monophasic and biphasic tumors or in relationship to immunophenotype.[9] In our case, the tumor was removed surgically, and postoperatively, patient was given chemotherapy and responded well to treatment. The patient is on follow-up and there is no recurrence reported yet.

RMS is a high grade, malignant mesenchymal tumor originating from primitive mesenchymal cells that can arise anywhere in the body. It is extremely rare in the retropharyngeal region. RMS is predominantly a disease of childhood and occurs somewhat less frequently in adolescents. The mean age at the diagnosis is 5–6 years and 72%–81% of patients are younger than 10 years.[10],[11] There is a slight male predominance, male: female being 1.3:1, whereas in our case, it was found in a young female patient aged 22 years, which is not in accordance with the previous studies.

Three main groups of RMS are recognized based on histological morphology: Embryonal, alveolar and pleomorphic, of which the former two mainly occur in children and the last only in adults.[12] Three different primary locations of head-and-neck RMS (HNRMS) are recognized: parameningeal (PM), nonparameningeal head and neck (NPM), and orbital (ORB) which are involved in approximately 50%, 25%, and 25% of cases, respectively.[13] Parameningeal location carries a worse prognosis compared to NPM and ORB locations.[13],[14] In our case, the location was nonparameningeal retropharyngeal, so the prognosis was better as compared to other locations.

Treatment of HNRMS requires a multidisciplinary approach where surgery, chemotherapy, and radiation therapy; each has its own specific role. Nonparameningeal tumors have favorable outcome. In our case, the patient underwent complete excision of the tumor followed by chemotherapy. Radiation was not advised as the tumor margins were free of disease. Prognosis appears to be strongly correlated with age, site, bones, and/or bone marrow involvement and the number of involved sites.[15] Our case did not have any distant metastasis at the time of presentation, which further improved the prognosis. At present, our patient is receiving chemotherapy, tolerating well, and showing no signs of recurrence.

The retropharyngeal space is a crucial space along the midline of the extracranial portion of the head and neck that extends from the skull base to the upper mediastinum. The disease process in the retropharyngeal space is extremely rare and uncommon. However, they assume greater significance because of the proximity of the retropharyngeal space to the airway and because of the inability to examine this space clinically.[16] Although rare, these entities should always be kept in mind, which could otherwise be missed easily even by a skillful surgeon. With early diagnosis and management of retropharyngeal tumors, the prognosis can be improved to a great extent.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Kransdorf MJ. Malignant soft tissue tumours in a large referral population: Distribution of diagnoses by age, sex and location. AJR Am J Roentgenol 1955;164:129-34.
2Miller RW, Young JL Jr., Novakovic B. Childhood cancer. Cancer 1995;75:395-405.
3Scully SP, Temple HT, Harrelson JM. Synovial sarcoma of the foot and ankle. Clin Orthop Relat Res 1999;(364):220-6.
4Ryan JR, Baker LH, Benjamin RS. The natural history of metastatic synovial sarcoma: Experience of the Southwest Oncology group. Clin Orthop Relat Res 1982;(164):257-60.
5Argani P, Faria PA, Epstein JI, Reuter VE, Perlman EJ, Beckwith JB, et al. Primary renal synovial sarcoma: Molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol 2000;24:1087-96.
6Fisher C. Synovial sarcoma. Am Diagn Pathol 1998;2:401-21.
7Chong VF, Fan YF, Khoo JB. Retropharyngeal lymphadenopathy in nasopharyngeal carcinoma. Eur J Radiol 1995;21:100-5.
8Fletcher CD, Unni KK, Mertens F, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of soft Tissue and Bone. Lyon, France: IARC Press; 2002. p. 200-4.
9Antonescu CR, Kawai A, Leung DH, Lonardo F, Woodruff JM, Healey JH, et al. Strong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcoma. Diagn Mol Pathol 2000;9:1-8.
10Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, et al. Intergroup rhabdomyosarcoma study IV: Results for patients with nonmetastatic disease. J Clin Oncol 2001;19:3091-102.
11Raney RB, Meza J, Anderson JR, Fryer CJ, Donaldson SS, Breneman JC, et al. Treatment of children and adolescents with localized parameningeal sarcoma: Experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through-IV, 1978-1997. Med Pediatr Oncol 2002;38:22-32.
12Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Merks JH. Imaging findings in noncraniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2008;38:617-34.
13Stevens MC, Rey A, Bouvet N, Ellershaw C, Flamant F, Habrand JL, et al. Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: Third study of International Society of Paediatric Oncology-SIOP Malignant Mesenchymal Tumour 89. J Clin Oncol 2005;23:2618-28.
14Oberlin O, Rey A, Anderson J, Carli M, Raney RB, Treuner J, et al. Treatment of orbital rhabdomyosarcoma: Survival and late effects of treatment– results of an international workshop. J Clin Oncol 2001;19:197-204.
15Oberlin O, Rey A, Lyden E, Bisogno G, Stevens MC, Meyer WH, et al. Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups. J Clin Oncol 2008;26:2384-9.
16Nyberg DA, Jeffrey RB, Brant-Zawadzki M, Federle M, Dillon W. Computed tomography of cervical infections. J Comput Assist Tomogr 1985;9:288-96.