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CASE REPORT
Ahead of print publication  

Carcinoma of the pyriform sinus with mediastinal metastasis successfully treated with chemoradiotherapy in resource-constraint setup


1 Department of Radiation Oncology, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Radiation Oncology, Tata Memorial Centre, Vishakhapatnam, Andhra Pradesh, india

Date of Submission07-Dec-2020
Date of Decision17-Jun-2021
Date of Acceptance25-Oct-2021
Date of Web Publication15-Jun-2022

Correspondence Address:
Pallavi Kalbande,
Department of Radiation Oncology, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_1667_20

 > Abstract 


Squamous cell carcinoma (SCC) of the pyriform sinus with metastatic mediastinal mass is staged as IVC and routinely treated with palliative intent. Here, we report a case cured with radical chemoradiotherapy without CT simulator, lead cutouts, and advanced techniques such as three-dimensional conformal radiation therapy, intensity-modulated radiation therapy, image-guided radiotherapy, and volumetric modulated arc therapy or stereotactic body radiotherapy. A 67-year-old male presented with SCC of the right pyriform sinus with mediastinal metastasis (Stage IVC). He was started with palliative chemotherapy afferent, but he could not tolerate it. Further, he was treated with radical chemoradiotherapy to dose of 60/30# to primary + neck with 6 MV photons and 50 Gy/25# to the anterior mediastinal lesion using 18 Mev electrons. Complete response to the treatment was achieved. At the close follow-up of 58 months, the patient is disease-free and follow-up is still ongoing. Limited metastatic disease can be completely cured using multimodality treatment using simple traditional 2D techniques, though optimal dose escalation becomes a limitation. Some variants of SCC do respond well even at suboptimal radiotherapy doses, so personalized treatment can be considered in such patients.

Keywords: Chemotherapy, metastasis, pyriform sinus, radiotherapy



How to cite this URL:
Kalbande P, Kale P, Ardha A. Carcinoma of the pyriform sinus with mediastinal metastasis successfully treated with chemoradiotherapy in resource-constraint setup. J Can Res Ther [Epub ahead of print] [cited 2022 Jul 2]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=347675




 > Introduction Top


Pyriform sinus is the most common subsite of hypopharyngeal tumors. Squamous cell carcinoma (SCC) of the pyriform sinus accounts for 70% of all hypopharyngeal cancer.[1] It carries one of the worst prognoses among head-and-neck cancers. Pyriform sinus is an area that allows tumors to grow silently. Thus, only a small number of patients present in early stage.[2],[3]

Distant metastasis from head-and-neck carcinomas is uncommon. The most common sites of distant metastasis are lung, liver, kidney, and adrenals. Distant metastasis in hypopharyngeal cancer to lung, mediastinum, and bone develops in 20%–40% of patients within 9 months of diagnosis and survival is usually <1 year after metastasis is detected.[4] In such patients of head-and-neck cancer with distant metastasis, a multimodality treatment approach is being adopted.

Patients with limited metastatic burden can be considered for radical treatment, provided curative radiotherapy doses delivered to primary and metastatic sites. 66-70Gy with concurrent chemotherapy is the optimal radiotherapy dose used for radical treatment in head-and-neck cancer. Limited literature is available on the management of carcinoma pyriform sinus with mediastinal metastasis. Mediastinum is a critical site to deliver radical radiotherapy doses because of dose-limiting structures such as lung and heart. It needs highly conformal techniques with accurate target delineation using computed tomography (CT)-based planning using intensity-modulated radiation therapy (IMRT), image-guided radiotherapy (IGRT), volumetric modulated arc therapy (VMAT), or stereotactic body radiotherapy (SBRT).

Radiotherapy department at our center is equipped with Clinac-ix without a CT simulator. Here, we report a case of SCC of the pyriform sinus with mediastinal metastasis completely cured using chemoradiotherapy in resource-constraint setup.


 > Case Report Top


A 67-year-old male patient presented in the outpatient department with complaints of dysphagia for solids for 1 month. It was associated with a change in voice. There was no history of pain, dyspnea, or weight loss. He was a chronic smoker with a 50 pack-year for 15 years and chronic alcoholic for 10 years. Flexible laryngoscopy under local anesthesia revealed a small 2.5 cm × 2 cm ulcerative lesion involving the right pyriform sinus extending up to the cricopharynx. The biopsy was taken from a visible lesion. The histopathological report came out to be well-differentiated SCC. Contrast-enhanced computed tomography (CECT) of the neck and chest showed an enhancing hypodense lesion of size 2.1 cm × 1.2 cm in the right pyriform sinus, inferiorly involved the right aryepiglottic folds [Figure 1]. Superiorly, there was no involvement of vallecula or epiglottis. Laterally, the fat plane with carotid was maintained, medially lesion was not crossing mid-line. Multiple well-defined hypodense oval lesions were noted in bilateral upper, middle, and lower jugular regions with largest of size 1.2 cm × 1.2 cm. In CECT of the thorax, an enhancing well-defined lesion of size 4 cm × 3.6 cm × 3.4 cm in anterior mediastinum on the left side was present. Fat plane between the lesion and aorta was maintained, but the fat plane between the lesion and left brachiocephalic trunk was lost. Suspicious mediastinal mass needed positron-emission tomography (PET)-CT scan to look for physiological uptake. However, PET CT was not available, so CT-guided fine-needle aspiration cytology was done, which came out to be metastatic SCC [Figure 2]. After complete diagnostic workup, the patient was staged as cT2N2CM1 (IVC) according to 2017 AJCC staging.
Figure 1: Pretreatment contrast-enhanced computed tomography showing well-defined enhancing lesion with irregular margins in anterior mediastinum on left site

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Figure 2: Fine-needle aspiration cytology from anterior mediastinal mass microscopy showing features of squamous cell carcinoma

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It was decided to treat this patient with palliative intent initially i/v/o metastatic disease. Palliative chemotherapy was given with nanopaclitaxel (100 mg D1) + cisplatin (30 mg D1-D5) +5 FU (820 mg D1-D5) according to departmental protocol. He could not tolerate the chemotherapy and developed grade three febrile neutropenia after the first cycle. The patient was managed inpatient conservatively. After recovery, it was decided to treat him with radical chemoradiotherapy with concurrent cisplatin 40 mg/m2 weekly, but it was a challenge to deliver radical dose with 2D technique. Three-dimensional conformal radiation therapy (3DCRT), IMRT, VMAT, IGRT, or SBRT could be the best and desirable to deliver a curative dose of 66–70 Gy at primary and critical metastatic sites with limiting normal tissue constraints. However unfortunately, none of them were available. It was a challenge to deliver radical dose with limited resources. Hence, we were obliged to treat with 2D technique using diagnostic CECT for metastatic and primary sites. He was planned for EBRT to the neck by 6 MV photons and to the anterior chest wall using 18 MeV electrons to total a dose of 66 Gy. For primary lesions, bilateral parallel opposed fields were planned to include regional lymph nodes. Radiotherapy dose of 66 Gy/33 fractions was prescribed to the mid-plane using a shrinking field technique. For mediastinal mass, single direct field was planned using a 6 by 6 cm electron applicator. To conform the electron field, lead cutouts were required, but it was also not available, so, complete field was treated without any shielding. Depth of treatment was decided from diagnostic imaging. Radiotherapy dose of 66 Gy/33 fractions was prescribed at the depth of 6 cm. The patient had successfully received radiotherapy of 60 Gy/30 fractions with four cycles of weekly cisplatin. He discontinued radiotherapy after 30 fractions due to unbearable acute toxicities, i.e. Grade III mucositis and Grade III skin reaction. 50 Gy/25 fractions could be successfully delivered to mediastinal lesion; further fractions were stopped to limit acute toxicity. For response assessment, CECT of the neck and chest was done 3 months after completion of treatment which has shown complete response of primary and metastatic lesions [Figure 3].
Figure 3: Posttreatment contrast-enhanced computed tomography scan showing smooth margins and no enhancement in anterior mediastinal lesion

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For confirmation of response, PET-CT scan was done from the higher center at Nagpur which has shown complete metabolic response [Figure 4]. The patient recovered gradually from acute toxicities and is on regular follow-up. At the end of the follow-up of 58 months, the patient is doing well with a good quality of life, there is no evidence of recurrence.
Figure 4: Posttreatment positron-emission tomography–computed tomography showing a complete metabolic response of mediastinal lesion

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 > Discussion Top


Carcinoma arising in the hypopharynx is uncommon with a high propensity to present in an advanced stage because of delayed symptoms. Regional nodal metastasis at the time of presentation is more common due to rich lymphatics of the hypopharynx. A small proportion of patients present with distant metastasis. Distance metastasis in SCC of head-and-neck cancer ranges from 4.3% to 30%.[5] The lung is the most common site of metastasis followed by bone and liver. Limited literature is available on the management of carcinoma of the pyriform fossa with an anterior mediastinal mass. The personalized treatment approach is recommended in such patients. In our patient, 3DCRT, IMRT, VMAT, IGRT or SBRT could be the best option for dose escalation using CT-based planning with PET-CT image fusion. Our radiotherapy department was equipped with Clinac-ix without CT simulator and telecobalt unit. It was challenging to treat him with limited resources. After treatment, we could not deliver radical radiotherapy doses to primary and mediastinal sites, and complete response was uncertain with suboptimal delivered doses.

Spector[6] has reported a 17.6% incidence of distant metastasis from carcinoma of the pyriform sinus. Five-year disease-specific survival was 6.4%. As the incidence of distant metastasis is low as compared to other cancer types such as breast and lung cancer, distant metastasis is a major determinant in prognosis and management.[7] Advancement in the management of patients with distance metastasis does not significantly improve overall survival in the past 20 years. Some studies have shown benefits for aggressive local therapy.[8] Oligo-metastatic patients were treated with surgery or SBRT depending on disease burden, Li et al. have reported poorer prognosis in metastatic patients.

In the present case, old age, advanced locoregional disease, and distant metastasis were the predictors of poor prognosis. Nonavailability of CT simulator, lead cutouts for electron beam, and advanced techniques such as 3DCRT, IMRT, VMAT, IGRT, and SBRT was a resource challenge to deliver a radical dose of 66–70 Gy with limiting normal tissue toxicity in critical area of anterior mediastinum. Patients with Stage IV head-and-neck cancer are routinely being treated with palliative approach and we had also started with the same. However, as he could not tolerate the chemotherapy, decision was taken to go with radical radiotherapy. After completion of chemotherapy, he could receive 60 Gy/30 fractions to the primary lesion and 50 Gy/25 fractions to the metastatic lesion with four cycles of weekly cisplatin. Despite the suboptimal dose received compared to radical dose, complete response was achieved. At the end of the close follow-up of 58 months, the patient is disease-free, and follow is still ongoing.


 > Conclusion Top


SCC of the hypopharynx is known to respond well with chemoradiotherapy. Limited metastatic disease can be completely cured using multimodality treatment even with 2D technique. A radical approach can be adopted in this presentation which is inherently considered palliative, and patients can be given a chance to be disease-free with good quality of life. Although delivering a radical dose is not always possible, some variants do respond well to sub-radical doses of radiotherapy and give satisfactory results in some patients.

Ethical statement

The patient confidentiality is maintained and prior informed consent was obtained from the patient.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Pingree TF, Davis RK, Reichman O, Derrick L. Treatment of hypopharyngeal carcinoma: A 10-year review of 1,362 cases. Laryngoscope 1987;97:901-4.  Back to cited text no. 1
    
2.
Elias MM, Hilgers FJ, Keus RB, Gregor RT, Hart AA, Balm AJ. Carcinoma of the pyriform sinus: A retrospective analysis of treatment results over a 20-year period. Clin Otolaryngol Allied Sci 1995;20:249-53.  Back to cited text no. 2
    
3.
Sewnaik A, Hoorweg JJ, Knegt PP, Wieringa MH, van der Beek JM, Kerrebijn JD. Treatment of hypopharyngeal carcinoma: Analysis of nationwide study in the Netherlands over a 10-year period. Clin Otolaryngol 2005;30:52-7.  Back to cited text no. 3
    
4.
Helliwell TR. Evidence based pathology: Squamous carcinoma of the hypopharynx. J Clin Pathol 2003;56:81-5.  Back to cited text no. 4
    
5.
Pérez-Hernández I, Sarroca-Capell E, Palomar-Asenjo V. Metástasis cutáneas en carcinoma de hipofaringe. Acta Otorrinolaringol Esp 2012;63:75-6.  Back to cited text no. 5
    
6.
Spector GJ. Distant metastases from laryngeal and hypopharyngeal cancer. Orl J Otorhinolaryngol Relat Spec 2001;63:224-8.  Back to cited text no. 6
    
7.
Li Y, Ou X, Hu C. Prevalence and prognostic impact of synchronous distant metastases in patients with hypopharynx squamous cell carcinomas: A SEER-based study. J Cancer 2019;10:620-6.  Back to cited text no. 7
    
8.
Lo SS, Moffatt-Bruce SD, Dawson LA, Schwarz RE, Teh BS, Mayr NA, et al. The role of local therapy in the management of lung and liver oligometastases. Nat Rev Clin Oncol 2011;8:405-16.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

 
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