Letters to editor

REFERENCES 1. Giardiello FM, Welsh SB, Hamilton SR, Offerhaus GJ, Gittelsohn AM, Booker SV, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med 1987;316:1511-4. 2. Hearle N, Schumacher V, Menko FH, Olschwang S, Boardman LA, Gille JJ, et al. Frequency and spectrum of cancers in the PeutzJeghers syndrome. Clin Cancer Res 2006;12:3209-15. 3. Pan SY, Morrison H. Epidemiology of cancer of the small intestine. World J Gastrointest Oncol 2011;3:33-42. 4. Choi HS, Park YJ, Youk EG, Yoon KA, Ku JL, Kim NK, et al. Clinical characteristics of Peutz-Jeghers syndrome in Korean polyposis patients. Int J Colorectal Dis 2000;15:35-8. 5. Mehta MV, Porecha MM, Mehta PJ. Small intestinal

adenocarcinoma in Peutz-Jeghers syndrome. Indian J Gastroenterol 2006;25:38-9. 6. Saranrittichai S. Peutz-Jeghers syndrome and colon cancer in a 10-year-old girl: Implications for when and how to start screening? Asian Pac J Cancer Prev 2008;9:159-61. Access this article online Quick Response Code:

Website: www.ijmpo.org

DOI:

10.4103/0971-5851.133741

Who will bell the cat? Sir, We read the editorial titled “Cat on a hot tin roof…” with interest.[1] This emotion filled article highlights the situation faced by many clinicians treating especially cancer patients.

work with hope in our hearts with the belief that sometimes miracles may happen.

According to the story, in our great epic Mahabharata, when the Yaksha asked the eldest Pandava, Yudhisthira “what is the most surprising event on earth?” Yudhisthira had supposed to answer that though every human being knows that death is inevitable still no one is ready to accept that eventually one will meet death.

Departments of Pathology, 1Surgical Oncology, and 2Medical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India. E-mail: [email protected]

It is distressing to see any person succumb to his/her illness. We firmly believe that the clinicians should be compassionate and sympathetic.[2,3] As stated in the article indeed there is a great need for counseling and palliative care. We are of the opinion that even with their demanding work schedule the clinicians should take the best possible care of their patients. Another thing is that we should have a healthy respect for the alternative medicine. May be in days to come we shall be equipped with more armament to fight this deadly ailment. Until then, we must

Rashmi Patnayak, Amitabh Jena1, N. Raghu Vamsi1, Dandugundu Bhargavi2

REFERENCES 1. Gupta S. Cat on a hot tin roof. Indian J Med Paediatr Oncol 2013;34:65. 2. Jena A, Patnayak R, Sampath VV. Tell the truth, but be gentle. South Asian J Cancer 2013;2:192. 3. Jena A, Patnayak R, Chowhan AK. Doctor as a healer. J Clin Sci Res 2013;2:119. Access this article online Quick Response Code:

Website: www.ijmpo.org

DOI:

10.4103/0971-5851.133742

Primary tonsillar Ewing sarcoma in young adult: A rare tumor at an uncommon site Sir, Extraosseous Ewing sarcoma is a rare malignant small round cell tumor that can potentially occur in any

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soft-tissue location. Common sites for Ewing sarcoma include lower extremities, paravertebral area of spine, retroperitoneum and chest wall. In head and neck

Indian Journal of Medical and Paediatric Oncology | Jan-Mar 2014 | Vol 35 | Issue 1

Letters to editor

regions, rarely extraosseous Ewing sarcoma from softtissue of nose, eyelid, nasopharynx, parotid gland, scalp and parapharyngeal spaces have been reported. [1] We report an unusual case of extraosseous Ewing sarcoma of tonsil. A 25-year-old male patient presented with 3 months history of dysphagia. The primary physician noted asymmetrical growth of left tonsil and was prescribed antibiotics with a presumptive diagnosis of tonsillitis. The symptoms persisted and he presented in emergency department at our hospital with odynophagia. Contrastenhanced computed tomography (CECT) scan of neck revealed 4 cm mass in tonsillar fossa extending into anterior tonsillar pillar, posterior oropharyngeal wall and parapharyngeal spaces [Figure 1a]. CECT scan of chest and abdomen showed multiple nodular lesions in both lungs [Figure 1b] and two metastatic lesions in left lobe of liver. An emergency tracheostomy was done and biopsy from tonsillar mass revealed sheets of regular, round to oval, small, undifferentiated cells [Figure 1c] which were immunopositive for CD99, negative for desmin, myogenin, leucocyte common antigen, CD34, chromogranin, synaptophysin, bcl2, epithelial membrane antigen and pan-cytokeratin [Figure 1d]. This was consistent with extraosseous Ewing sarcoma although we were not able to perform

the test for chromosomal translocation t(11;22). Bone scan revealed multiple bone metastases while the bone marrow biopsy did not reveal any tumor deposits. He was started on combination chemotherapy including vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide with palliative intent. Post 4 cycles of chemotherapy, response evaluation revealed stable disease. He was continued on chemotherapy but died due to disease progression after 6 cycles. Unilateral tonsillar enlargement is considered to be a sign of malignancy. Primary malignant tumor of tonsil represents 10% of oral cavity neoplasms. The most common malignancy of tonsil are squamous cell carcinoma and non-Hodgkin lymphoma and rare tumors of tonsil include extramedullary plasmacytoma, Hodgkin lymphoma and metastatic deposits.[2] There has been one previous report of non-metastatic tonsillar Ewing sarcoma in a 75-year-old male.[3] The above case is the first case of tonsillar Ewing sarcoma presenting with metastases and is an alert for head and neck physicians and teen and young adult oncologists to be aware of this rare tumor at this uncommon site. Ajay Gogia, Lavleen Singh1, A. K. Karak1, Sameer Bakhshi Departments of Medical Oncology, and 1Pathology, Dr. B. R .A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India E-mail: [email protected]

REFERENCES 1. a

b

c

d

Figure 1: Contrast-enhanced computed tomography (CECT) scan of neck revealed well-defined homogenous soft-tissue mass 4 cm mass in tonsillar fossa extending into anterior tonsillar pillar and oropharyngeal wall (a); CECT scan of chest showed nodular lesions in right upper lobe of lung (b); biopsy of tonsil showing a sheet of regular, malignant round small undifferentiated cells with frequent mitoses (H and E, ×200) (c); tumor cells show diffuse membranous immunopositivity for mic-2. (H and E, ×400) while they are immunonegative for bcl-2 (inset) (d)

Ng SH, Ko SF, Cheung YC, Wong HF, Jung SM. Extraskeletal Ewing’s sarcoma of the parapharyngeal space. Br J Radiol 2004;77:1046-9. 2. Cortez EA, Mattox DE, Holt GR, Gates GA. Unilateral tonsillar enlargement. Otolaryngol Head Neck Surg (1979) 1979;87:707-16. 3. Shao CH, Huang SC, Hwang CF, Peng JP. Extraosseous Ewing sarcoma of the tonsil. J Otolaryngol Head Neck Surg 2012;41:E35-7. Access this article online Quick Response Code:

Indian Journal of Medical and Paediatric Oncology | Jan-Mar 2014 | Vol 35 | Issue 1

Website: www.ijmpo.org

DOI:

10.4103/0971-5851.133743

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Primary tonsillar Ewing sarcoma in young adult: A rare tumor at an uncommon site.

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