Volume 3, Issue 1 , Pages 44-47, January 2008
Nasal ala rhabdomyosarcoma misdiagnosed as a facial infection
Article Outline
Summary
We present the case of an 18-month-old female with nasal ala swelling thought to be of infectious etiology. There was no response to multiple courses of oral and topical antibiotics. Biopsy of the lesion revealed alveolar rhabdomyosarcoma. The patient received the D9803 closed trial chemotherapy protocol for intermediate risk rhabdomyosarcoma standard arm and high-dose-rate brachytherapy. Treatment with a combination of chemotherapy and brachytherapy is becoming increasingly accepted for this type of tumor and is associated with limited morbidity. Although facial rhabdomyosarcoma is relatively uncommon, it should be part of the differential diagnosis in the presence of a rapidly growing facial swelling unresponsive to antibiotics.
Keywords: Rhabdomyosarcoma, Head and neck oncology, Pediatric oncology, Nasal ala, Facial infection, Brachytherapy
1. Case Report
A previously healthy 18-months-old female presented to her paediatrician after her parents had noticed facial asymmetry and enlargement of the left nostril. She was given a course of oral antibiotics and a referral to otolaryngology in the event that the swelling would not subside. One month later, as the swelling had not regressed, the patient presented to an otolaryngologist. The swelling was now involving her left nostril and nasal vestibule and the overlying skin was discoloured. She was prescribed Fuciderm, a topical antibiotic and the parents were warned that the lesion might require biopsy. The sinus and nasal X-rays were negative. Two weeks later she was seen by a different otolaryngologist who thought the lesion was infectious and prescribed Fucidin, a topical antibiotic. A week later, as there was no response the parents were referred to the senior author (SJD). By this time, the lesion had progressed to a 1
cm×1cm, ill-defined, firm, non-tender swelling involving her left nostril, and the lateral side of the bony pyramid (See Fig. 1). A biopsy was performed and the pathology revealed rhabdomyosarcoma of the alveolar subtype. Genetics studies revealed consensus primers to be positive for t(2;13)(q35;q14). An iliac crest bone marrow biopsy showed no evidence of metastasis and the results of a bone scan were normal. Computer tomography (CT) of the chest and the neck were performed. The chest CT was normal while the neck CT showed a fairly well circumscribed ovoid 2.1cm×1.8cm×1.2cm region of intermediate soft tissue density the region of the left nasal ala extending from the inferolateral edge of the left nasal bone down to just superolateral to the filtrum on the left side (Fig. 2). There was also mild stranding of the superficial fat at the bridge of the nose, but no definite extension across midline and the deep tissues were spared. The neck CT also revealed several prominent lymph nodes, mostly at the anterior and cervical chains bilaterally, but most importantly on the left side. It was questionable whether the enlarged lymph nodes were due to an inflammatory cause secondary to a sinusitis or whether they were malignant.
Fig. 1.
Picture demonstrating the patient with rhabdomyosarcoma of the left nasal ala. Notice the marked asymmetry of the ala.
Fig. 2.
Enhanced CT scan of the face shows soft tissue density mass (2.1
cm×1.8cm×1.2cm) seen involving the region of the left nasal ala without bony invasion.
The patient received the D9803 closed trial chemotherapy protocol for intermediate risk Rhabdomyosarcoma standard arm, which consists of vincristine, dactinomycin and cyclophosphamide, administered over 39 weeks. She also underwent a 5-day course of high-dose-rate brachytherapy (Fig. 3).
Fig. 3.
Follow-up CT scan demonstrates brachytherapy catheter in situ, with good response to therapy.
2. Discussion
Rhabdomyosarcoma is a malignant neoplasm derived from embryonic mesenchymal tissue expressing varying degree of striated muscle cell differentiation. It is mainly a juvenile neoplasm, with 70% presenting in children younger than 12 years old [1]. The two most common histological subtypes in childhood are embryonal and alveolar. About 35% of cases of rhabdomyosarcoma arise in the head and neck [1] with a quarter of these presenting in non-parameningeal non-orbital locations [2].
Rhabdomyosarcoma presenting in the face can easily be initially mistaken for an abscess or an infection. However, their unresponsiveness to antibiotics or incision and drainage and their continuous growth eventually leads to a biopsy and diagnosis of the neoplasm. The prognosis of rhabdomyosarcoma presenting in this region is generally favourable, as the tumor tend to become visible early, thus pushing patients or their caregivers to seek medical attention when the neoplasm is still at an early stage. For head and neck rhabdomyosarcoma a tumor size smaller than 5cm and age younger than 11 years old are associated with a significantly better outcome [3].
While chemotherapy and radiotherapy with or without surgery used to be the standard treatment for rhabdomyosarcoma, brachytherapy is becoming increasingly used for head and neck tumors. Brachytherapy's major advantage is its tissue-sparing approach, which is especially important in young children. Other non-negligible advantages over radiotherapy include the elimination of radiation to the caregiver of young children necessitating immobilization during the treatment, a reduced treatment time and a decreased bone marrow radiation [4]. There are, however, some downsides to brachytherapy. First, the treatment being much more localized, there is an increased risk of missing part of the tumor. In addition, the treatment is inhomogeneous and may thus cause increased morbidity in certain areas and under-treatment in other critical areas. In an analysis of the failures of the AMORE (ablative surgery, moulage technique brachytherapy, and surgical reconstruction) protocol [5], it was suggested that local relapse was related to incomplete surgery and suboptimal position of the mold used to deliver brachytherapy. In a study of 15 children suffering from soft tissue sarcoma, 12 of which suffering from rhabdomyosarcoma, treatment with a combination of chemotherapy, surgery and fractioned high-dose brachytherapy [4] was shown not to cause excessive morbidity, compromise in local control or increase in disease-specific survival. However, three patients did suffer from grade three or four morbidity due to brachytherapy. The conclusions of this study were that in older children who can understand the need for immobilization and tolerate hospitalization low-dose-rate treatment might be preferable, while in younger children high-dose-rate brachytherapy is most beneficial.
3. Conclusion
Although rhabdomyosarcomas presenting as a swelling in the region of the nasal ala is rare, it should be kept in mind when a child present with localized facial swelling unresponsive to antibiotic therapy. Because of the location, patients with such tumors tend to seek medical care early and are diagnosed at an early stage when the prognosis is favourable. Due to its more localized action, brachytherapy is increasingly replacing radiotherapy for head and neck rhabdomyosarcoma, however data on its effectiveness are still limited.
References
- . Pediatric sinonasal rhabdomyosarcoma: three cases and a review of the literature. Am. J. Otolaryngol. 2003;24(3):174–180
- . Rhabdomyosarcoma of the head and neck in children: the experience at the Children's Hospital of Philadelphia. Med. Pediatr. Oncol. 1986;14:288–292
- . Prognostic factors in head and neck rhabdomyosarcoma. Head Neck. 2002;24:468–473
- . Long-term morbidity in children treated with fractioned high-dose-rate brachytherapy for soft tissues sarcomas. J. Pediatr. Hematol./Oncol. 2003;25(6):448–452
- AMORE protocol in pediatric head and neck rhabdomyosarcoma: descriptive analysis of failure patterns. Head Neck. 2005;27:390–396
PII: S1871-4048(07)00078-0
doi:10.1016/j.pedex.2007.10.001
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 1 , Pages 44-47, January 2008
