January 2007 Vol 7 correct A.indd Rhabdomyosarcoma of the Nasal Vestibule in a Child George H Tanyous About 5-8% of childhood tumours are Rhab-domyosarcomas (RM).1 A sarcoma is a malig-nancy originating from primitive mesenchy- mal cells, which under normal circumstances develops in supportive tissues such as muscle and bone. T H E C A S E A two year old girl was brought in the Ear Nose Throat (ENT) Department of SQUH by her parents with a mass blocking the right anterior nares completely, causing difficulty in breathing. The blockage had lasted already several weeks. There were no other symptoms like itching or bleeding. On examination, the child looked healthy, but had a spherical mass about 1cm x 1cm in diameter attached to the skin of the floor of the nasal vestibule. This was covered with intact skin, which although it looked pink had no ulceration or tenderness. The mass was not fixed to the surrounding tissue. There were no palpable cervical glands. The left nasal cavity and the oral cavity were normal. On admission and after a complete blood count and blood chemistry all results were found to be normal. It was obvious that an excision biopsy would pro- vide the correct diagnosis. This was done under gen- eral anesthesia with an oro-tracheal intubation and a pharyngeal pack. The swelling was actually shaved out of the nasal vestibule taking care not to injure the car- tilage of the vestibule. The bleeding was very mild and quickly stopped by application of pressure and a small anterior nasal pack. The specimen was sent to the his- topathology department. Postoperatively, the child made an uneventful recov- ery. The nasal pack was removed after 24 hours and the denuded area of the vestibule start to heal well without problems. The area was painted daily with antiseptic cream and the child was given prophylactic antibiotic for seven days (Augmentin syrup). The specimen was sent to the histopathological department and the result came as RM of the alveolar type with positive mono- clonal antibodies to desmin (tumour related antigen). The child was referred to the pediatric oncologist and was prescribed chemotherapy. It is well known that RM is chemosensitive, with the use of multiple drugs (5) nor- mally required. The intensity of treatment depends on 3 Cooliban St., Merylands West, New South Wales, 2160 Australia Email: scarface300@hotmail.com طفل أَنْف ليزُ دِهْ في ة طَ طَّ َ ُا لِيَّةُ ضَ العَ اركومةُ السَّ على حالتهاالسريرية تؤشر ال . حلصوله مسبق ب ؤَهِّ مُ عامل وجود بدون سنتني بعمر ة في طفلة طَ َطَّ ُلِيَّةُ ا ضَ العَ اركومةُ السَّ امللخص: نقدم حالة يّة. نْخِ السِ ة طَ َطَّ ُا لِيَّةُ ضَ العَ اركومةُ هو السَّ املرضي النسيجي الفحص تقرير كان . اتبر من كمفاجأة جاء الذي تشخيص املرض . عمان ، حالة تقرير ، الطفولة ، االنفية املفردات املفتاحية: الساركومة العضلية اططة ABSTRACT A case of rhabdomyosarcoma in a 2 year old girl without a pre-existing predisposing factor vis- ited the ENT Department of Sultan Qaboos University Hospital (SQUH). Her clinical condition did not point to the diagnosis, which came as a histological surprise. The histopathology report was alveolar rhabdomyosarcoma. Keywords: Nasal rhabdomyosarcoma, pediatric age, Case Report, Oman. SULTAN QABOOS UNIVERSITY MEDICAL JOURNAL DECEMBER 2006 VOL 6, NO. 2 SULTAN QABOOS UNIVERSITY© C A S E S T U D Y G E O R G E H TA N Y O U S 88 - The stage of the sarcoma - The site - The histopathology The child initially recovered well, however she did not report one month later for the usual follow up. D I S C U S S I O N Most sarcomas show some evidence of differentiation into a particular supportive tissue, but maturation is incomplete. The most common normal tissue counterpart for childhood sarcomas is skeletal muscle, and tumors having features of skeletal muscle differentiation are called rhabdomyosarcomas. A small subset of children with rhabdomyosar- comas, mostly genito-urinary rhabdomyosarcomas, belong to families in which multiple cancers such as breast lung and brain tumors occur. Adrenocortical cancer and leukemia can also develop. Constitutional mutation in the p53 can be found. The tumor suppres- sor gene located on the long arm of chromosome 17, characterizes this cancer predisposition syndrome (the Li Fraumeni Syndrome). RM arise from mesenchymal tissue. The head and neck region is the most common site of origin in 40% of cases, the genito-urinary in 20% of cases, 20% in the extremities and 10% in the trunk.6 RM is the most common type of head and neck cancer in the first 2 years of life.1 50% of head and neck tumors arise in the orbit and the nasopharynx. SUBD I V I SI O N 25% arise in the orbit, 50% in non-orbital paramenin- geal sites (the nasopharynx, middle ear, mastoid and pterygoid and infra temporal fossa) 25% are non-or- bital non-parameningeal (the mouth, the neck, face, scalp, larynx).2 Symptomatology It usually presents as a painless mass. In the larynx it results in upper airway obstruction. In a few cas- es there is persistent pain with sanguinous discharge and cranial nerve palsies. In the areas covered with mucous membrane it can have a botryoid (bunch of grapes) appearance. Diagnosis This can be done by MRI scan set at bone windows and/or biopsy. Histopathology Spindle shaped cells with eosinophlic cytoplasm or cross striation like striated muscles. However, the most common appearance is that of an undifferentiated small, round, blue cell tumour with the differential di- agnosis of neuroblastoma, Ewing’s sarcoma, primitive neuroectodermal tumour or non Hodgkin’s lympho- ma. Monoclonal antibodies which are directed against the tumor-related antigens, such as desmin in RM or leucocytes common antigen (in case of lymphoma) or neuron specific enolase (in the case of neuroblastoma or primitive neuroectodermal tumors), can help in a differential diagnosis. Pathological sub-types 1. Embryonal (botryoid) variant with good progno- sis, arising in a sub-mucosal location. 2. Alveolar with poor prognosis, due to aggressive behaviour and tendency to metastasize early. 3. Pleomorphic, the most common in adults, rare in children Spread The tumor spreads to regional lymph nodes in 5% of cases and to lung, liver, bone and bone marrow, with 4 stages of tumor according to the degree of spread. Treatment 1. Surgical complete excision or biopsy. However, successful complete excision (biopsy) is normally achieved in only 4 out of 60 patients. 2. Multiagent chemotherapy, judicious surgery and radiotherapy to selected patients 3. Chemotherapy (chemo sensitive) with cyclophos- phamide and ifosfamide. R H A B D O M Y O S A R C O M A O F T H E N A S A L VE S T I B U L E I N A C H I L D 89 Actinomycin D, vincristine, Doxorubicin C, Toposide, platinum analogues like cis and carboplatinum. At least 3 drugs should be given. Prognosis 1. 5 years survival: 92% with in orbital RM. 2. 81% with non-parameningeal non-orbital. 3. 69% with parameningeal. R E F E R E N C E S 1. Anderson, G J, Tom L W C, Womer R B, Handler S D, Wetmore R F, Potsic W P. Rhabdomyosarcoma of the Head and Neck in children. Arc Otolaryngol Head Neck Surg 1990; 116:428-431. 2. Kato M A D P, Flamant F, Terrier-Lacombe M J, Habrand J L, Schwaab G, Luboinski B, et al. Rhabdomyosarcoma of the larynx in children: a series of five patients treated in the Institut Gustave Roussy (Villejuif, France). Medi- cal and Pediatric Oncology 1991; 19:110-114. 3. Kilman J W, Clatworthy H W, Newton W A, Grosfeld J W. Reasonable surgery for rhabdomyosarcoma: a study of 67 cases. Ann Surgery 1993; 178:346-351. 4. Maurer H M, Gehan E A, Beltangady M, et al. The Inter- group Rhabdomyosarcoma Study - II. Cancer 1992; 71:1904-1922. 5. Stevens M, Oberlin O, Flamant F, Rey A, Praquin M T. SIOP MMT 89 protocol for non-metastatic rhabdomy- osarcoma - An update. Medical and Paediatric Oncol- ogy 1993; 21:554. 6. Corbett R, Printh J, Plowman PN. Tumours of the Head and Neck. In: Adams DA, Cinnamond MJ, editors. Paediatric Otolaryngology. Series: Kerr AG Ed. Scott Brown Otolaryngology. 6th Ed. Oxford Butterworth Heinemann, 1977: 6/31/6-9. 7. Herrmann B W, Sotelo-Avila C, Eisenbeis J F. Pediatric sinonasal rhabdomyosacrcoma: three cases and a review of the literature. Am J Otolaryngol 2003; 24:174-80