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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 39  |  Issue : 2  |  Page : 94-97

Sarcomatoid carcinoma of the pyriform sinus: A rare malignancy in a rare location responsive to combination of chemotherapy and radiotherapy


1 Department of Radiation Oncology, Command Hospital (Southern Command), Pune, Maharashtra, India
2 Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India
3 Department of ENT Head and Neck Onco-Surgery, Command Hospital (Southern Command), Pune, Maharashtra, India
4 Department of Nuclear Medicine, Command Hospital (Southern Command), Pune, Maharashtra, India

Date of Submission24-Aug-2018
Date of Decision14-Oct-2018
Date of Acceptance30-Nov-2018
Date of Web Publication28-Mar-2019

Correspondence Address:
Dr. Abhishek Purkayastha
Department of Radiation Oncology, Command Hospital (Southern Command), Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmedsci.jmedsci_131_18

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  Abstract 

Sarcomatoid carcinoma (SaC) is a rare variant of squamous cell carcinoma with sarcomatoid features having biphasic characteristic malignant mesenchymal spindle cell and squamous cell component comprising of dysplasia, carcinoma in situ, or invasive carcinoma. Most information about this malignant entity in the head-and-neck region has emerged from larynx and oral cavity with an incidence of 0.5%–3% and 1%, respectively, with very few cases reported in hypopharynx. We hereby report such a case in a 76-year-old male who presented with dysphagia and swelling right side neck. Clinicopathological evaluation showed a localized SaC hypopharynx. In view of old age and comorbidities of hypertension and diabetes, no surgical intervention was contemplated, and he was treated with definitive concurrent chemo-radiotherapy (CCRT). A thorough review of the literature revealed that while earlier reported cases underwent upfront surgery followed by adjuvant RT or adjuvant CCRT, we treated this case with combination of chemotherapy and RT resulting in a significant therapeutic effect.

Keywords: Sarcomatoid carcinoma, hypopharynx, pyriform sinus, chemotherapy, radiotherapy


How to cite this article:
Purkayastha A, Singh S, Bisht N, Shelly D, Gupta V, Husain A. Sarcomatoid carcinoma of the pyriform sinus: A rare malignancy in a rare location responsive to combination of chemotherapy and radiotherapy. J Med Sci 2019;39:94-7

How to cite this URL:
Purkayastha A, Singh S, Bisht N, Shelly D, Gupta V, Husain A. Sarcomatoid carcinoma of the pyriform sinus: A rare malignancy in a rare location responsive to combination of chemotherapy and radiotherapy. J Med Sci [serial online] 2019 [cited 2019 Apr 18];39:94-7. Available from: http://www.jmedscindmc.com/text.asp?2019/39/2/94/248494


  Introduction Top


Sarcomatoid carcinoma (SaC) is a rare malignancy with unique biphasic histologic pattern reported in various organ systems[1] apart from head-and-neck (HN) with larynx being the most common location[2] with an incidence of 0.5%[3]–3%[4],[5] followed by oral cavity with 1%.[6] HN SaC is associated with a dismal prognosis as compared to extra-oral sites with higher recurrence and mortality due to extensive lymphatic and distant dissemination.[1] Surgery has been considered the standard of care for HN SaC[4] but concurrent chemoradiotherapy (CCRT) as a definitive therapeutic modality may result in excellent clinical and radiological response, as seen in our case.


  Case Report Top


A 76-year-old male, chronic smoker, hypertensive and diabetic presented with dysphagia and swelling right side neck. Clinical examination showed conglomerate lymph node (LN) mass right neck cervical level II/III. Otorhinolaryngologic examination showed a 3.8 cm × 2.5 cm growth right pyriform sinus (PFS), biopsy from which revealed pleomorphic spindle cell tumor with large hyperchromatic nuclei, prominent nucleoli with frequent mitosis with a mitotic rate of 4 per high-power-field arranged in intersecting fascicles. Few strips of squamous mucosa showing full thickness dysplasia were also present [Figure 1]. Immunohistochemistry (IHC)-stained positive for pan-cytokeratin (pan-CK) [Figure 2], p63 [Figure 3] and S-100 [Figure 4] while stained negative for smooth muscle actin (SMA) which gave a diagnosis of SaC of PFS.
Figure 1: Biopsy from right pyriform sinus showing pleomorphic spindle cell tumor with large hyperchromatic nuclei, prominent nucleoli with frequent mitosis with a mitotic rate of 4 per high-power-field arranged in intersecting fascicles. Few strips of squamous mucosa showing full thickness dysplasia was also present (a) H and E, ×10; (b) H and E, ×40

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Figure 2: Immunohistochemistry staining positive for pan-cytokeratin (pan-CK) (×10)

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Figure 3: Immunohistochemistry staining positive for p63 (×10)

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Figure 4: Immunohistochemistry staining positive for S-100 (×10)

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Whole body 18-fluorodeoxyglucose positron emission computed tomography (WB-18-FDG-PET/CT) scan showed a 2.4 cm × 1.1 cm × 1.1 cm growth right PFS [Figure 5] with a standard uptake value (SUV) of 10.36 and LNs 6.2 cm × 4.2 cm right level II and 3.5 cm × 2.6 cm right level III with SUV of 19.99 and 8.81, respectively [Figure 6]. Given old age, multiple comorbidities and N3 nodal status with fixity to underlying structures indicating an advanced stage, no active surgical intervention was contemplated. He was treated with external beam RT to a dose of 70 Gy in 35 fractions along with 5 cycles of 50 mg weekly cisplatin given on a concurrent basis at 40 mg/m2. Due to persistent nausea, vomiting, and leukopenia, the patient was not given the sixth chemotherapy cycle and also no further adjuvant chemotherapy was planned or given. He showed significant therapeutic benefit to treatment with improvement in dysphagia and complete resolution of the LN mass [Figure 7]. Clinical evaluation and WB-18-FDG-PET/CT 3 months post-CCRT showed complete resolution of the PFS growth along with cervical LNs [Figure 8]. At present, under follow-up and is disease free for more than 9 months.
Figure 5: Whole body 18-fluorodeoxyglucose positron emission computed tomography scan showing growth right pyriform sinus (blue pointer)

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Figure 6: Conglomerate lymph node mass right neck cervical level II/III (a) sagittal section; (b) axial section (yellow pointer)

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Figure 7: (a) Patient at initial presentation with swelling left side neck, (b) Patient showing significant therapeutic benefit with improvement in dysphagia and complete resolution of the lymph node mass post-chemo-radiation

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Figure 8: Follow-up Whole body 18-fluorodeoxyglucose positron emission computed tomography scan 3 months post concurrent chemo-radiotherapy showing complete metabolic resolution of the growth along with cervical lymph nodes

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  Discussion Top


SaC is a rare form of squamous cell carcinoma (SqCC) consisting of an epithelial component with poorly differentiated squamous and a mesenchymal component with spindle cell proliferation[1],[2],[3],[4],[5] with both components arising monoclonally from a single stem cell.[5] The sarcomatoid component represents a dedifferentiation from the squamous cells and known by various names as spindle cell carcinoma, carcinosarcoma, or pseudosarcoma.[1],[5] Apart from HN, SaC has been reported in most commonly in lung followed by urinary bladder, upper aero-digestive tract, breast, gallbladder, small intestine, colon, ovary, and prostate.[1] Larynx is the most common location in HN SaC[1],[2],[3],[4],[5] with the most common sub-site being glottis followed by buccal mucosa in oral cavity.[2],[5] Hypopharynx as an epicenter for HN SaC has been very rarely reported and to name a few; Viswanathan et al.[5] reporting six cases, Sharma et al.,[7] 4 cases in Indian literature while Chang et al.,[1] reported 8 cases the highest till date, Iqbal et al.,[4] 2 cases, Terada[8] 1 case, Zheng et al.,[9] 2 and Hardisson et al.,[10] reported 1 case.

HN SaC has been reported mainly in elderly males[1],[2],[5] between 5th and 7th decades of life,[2],[5] with risk-factors such as tobacco chewing in oral cavity,[1],[2],[5] smoking and alcohol consumption in larynx[1],[2],[3],[4],[5] and hypopharynx,[4],[5],[6],[7],[8],[9],[10] and prior RT exposure.[1],[5] Early stage lesions have been mostly reported in Western literature[2] as compared to advanced stages reported in developing countries[5] due to late reporting of symptoms and lack of advanced diagnostic facilities. SaC oral cavity mostly presents as ulceroproliferative growth associated with trismus and pain,[5],[6] laryngeal lesions with hoarseness of voice while hypopharyngeal lesions cause dysphagia, odynophagia, and foreign body sensation.[5],[7],[8],[9],[10] Lymph node (LN) involvement is most common in oral SaC[1],[5] while least common in hypopharyngeal lesions with an incidence of 0.5%,[5] though our case presented with N3 LN. However, as compared to HN SqCC, SaC has been found to have a higher lymphatic involvement as it can spread more easily within tissue planes which further increases the risk of distant dissemination.[1] Dissemination from hypopharynx has been to brain and skin[10] while other HN sites spread to lung, liver, bone, soft tissue, skin and heart.[1]

SaC has a biphasic nature comprising of the sarcomatous spindle cells which are arranged in various patterns such as fascicles as seen in our case and also storiform pattern[2],[5] and epithelial component.[1],[2],[5] Tumor cells show prominent eosinophillic nucleus with high mitotic counts of 3–8/hpf with bizarre atypical mitosis.[5] The epithelial component may be in the form dysplasia or carcinoma-in-situ or component of the infiltrating tumor. IHC differentiates a SaC from other malignant mesenchymal tumors such as leiomyosarcoma, myoepithelial carcinoma, mucosal spindle cell melanoma, and osteosarcoma. IHC strongly positive for epithelial markers such as pan-CK and p63 and weekly positive for S-100 confirms the diagnosis of SaC while negativity for SMA, desmin, vimentin, calporin, epithelial membrane antigen (EMA) rules out other pathologis.[2],[5] Due to rarity, the debate regarding its optimal management continues. Few authors have used upfront surgery (near total hypopharyngectomy) followed by adjuvant CCRT[9] or only RT[1],[9] while we exhibited upfront CCRT.


  Conclusion Top


Attempts to understand the development, progression, and management of this tumor have been non-beneficial due to the lack of large clinical trials. Identification of unique chromosomal abnormalities, biologic pathways are necessary for the sequencing of events associated with its evolution and the development of novel biological targets for therapy. SaC should always be considered in patients presenting with a PFS lesion so as to enable a prompt initiation of appropriate treatment. We also emphasize the role of CCRT in all cases of PFS lesions with a significant clinical benefit without the morbidity of extensive surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgment

We would like to thank the patient for allowing us to publish the case report and use the images taken during her stay in hospital.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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
    
2.
Thompson LD, Wieneke JA, Miettinen M, Heffner DK. Spindle cell (sarcomatoid) carcinomas of the larynx: A clinicopathologic study of 187 cases. Am J Surg Pathol 2002;26:153-70.  Back to cited text no. 2
    
3.
Dubal PM, Marchiano E, Kam D, Dutta R, Kalyoussef E, Baredes S, et al. Laryngeal spindle cell carcinoma: A population-based analysis of incidence and survival. Laryngoscope 2015;125:2709-14.  Back to cited text no. 3
    
4.
Iqbal MS, Paleri V, Brown J, Greystoke A, Dobrowsky W, Kelly C, et al. Spindle cell carcinoma of the head and neck region: Treatment and outcomes of 15 patients. Ecancermedicalscience 2015;9:594.  Back to cited text no. 4
    
5.
Viswanathan S, Rahman K, Pallavi S, Sachin J, Patil A, Chaturvedi P, et al. Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: A clinicopathologic review of 103 cases from a tertiary referral cancer centre. Head Neck Pathol 2010;4:265-75.  Back to cited text no. 5
    
6.
Mahajan A, Mohanty S, Ghosh S, Urs AB, Khurana N, Gupta S, et al. Sarcomatoid carcinoma of the oral cavity: A diagnostic dilemma. Case Rep Dent 2017;2017:7495695.  Back to cited text no. 6
    
7.
Sharma A, Das R, Sharma JD, Kataki AC. Spindle cell carcinoma of the head and neck: A clinicopathological and immunohistochemical study of 40 cases. J Cancer Ther 2012;3:1055-9.  Back to cited text no. 7
    
8.
Terada T. Sarcomatoid squamous cell carcinoma of pharynx: A case report with immunohistochemical study. Case Rep Clin Pathol 2016;3:39-42.  Back to cited text no. 8
    
9.
Zheng Y, Xiao M, Tang J. Clinicopathological and immunohistochemical analysis of spindle cell carcinoma of the larynx or hypopharynx: A report of three cases. Oncol Lett 2014;8:748-52.  Back to cited text no. 9
    
10.
Hardisson D, De Diego JI, Prim MP. Sarcomatoid carcinoma of the pyriform sinus with brain and subcutaneous metastases: An unusual metastatic spread pattern. Eur Arch Otorhinolaryngol 2000;257:445-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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