Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2019  |  Volume : 24  |  Issue : 3  |  Page : 212--215

Pediatric tracheal rhabdomyosarcoma masquerading as a granuloma


Marcus Hershey1, Casie M James1, Maria Velez2, Jhanvi Kanotra3, Randall Craver4, Sohit Paul Kanotra5,  
1 Department of Otolaryngology-Head and Neck Surgery, Louisiana State University, Louisiana, USA
2 Section of Hematology and Oncology, Children's Hospital New Orleans, Louisiana State University, Louisiana, USA
3 Department of Pathology, Louisiana State University, Louisiana, USA
4 Department of Pathology and Pediatrics, Children's Hospital New Orleans, Louisiana State University Health Science Center, Louisiana, USA
5 Department of Otolaryngology-Head and Neck Surgery, Louisiana State University; Department of Pediatric Otolaryngology, Children's Hospital New Orleans, Louisiana, USA

Correspondence Address:
Dr. Sohit Paul Kanotra
Department of Otolaryngology-Head and Neck Surgery, Pediatric Aerodigestive Center, Children's Hospital New Orleans, Louisiana State University, 200 Henry Clay Avenue Suite 4119, New Orleans, 70118 Louisiana
USA

Abstract

We describe a posterior wall intratracheal embryonal rhabdomyosarcoma (RMS) arising in a 6-year-old tracheostomized child masquerading as reactive granulation tissue and review all reported cases of pediatric intratracheal RMS. The child underwent laser debulking of the tumor and postoperative radiation and chemotherapy with no evidence of recurrence at 2-year follow-up. A literature review revealed four previous cases of pediatric primary tracheal or intratracheal RMS, and remission was achieved in all but one case with surgery, chemotherapy, and radiation. Pathologic evaluation of tracheal mucosal granulation tissue may merit consideration, particularly in patients with increased risk factors.



How to cite this article:
Hershey M, James CM, Velez M, Kanotra J, Craver R, Kanotra SP. Pediatric tracheal rhabdomyosarcoma masquerading as a granuloma.J Indian Assoc Pediatr Surg 2019;24:212-215


How to cite this URL:
Hershey M, James CM, Velez M, Kanotra J, Craver R, Kanotra SP. Pediatric tracheal rhabdomyosarcoma masquerading as a granuloma. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2019 Sep 20 ];24:212-215
Available from: http://www.jiaps.com/text.asp?2019/24/3/212/259746


Full Text



 Introduction



Rhabdomyosarcoma (RMS) is a soft-tissue sarcoma of mesenchymal origin, which represents a rapidly proliferating tumor that may readily invade surrounding tissues and metastasize to distant organs. While RMS represents <1% of solid tissue malignancies in adults, they comprise approximately one-half of all pediatric soft-tissue sarcomas and are the most common soft-tissue sarcoma in children. The 5-year survival rate for soft-tissue sarcomas in all patients has increased from 25% to 73% over the last few decades.[1] The two main RMS subtypes are embryonal (ERMS) and alveolar subtypes, most commonly involving the head and neck and the genitourinary system.[1] Only four tracheal RMS have been reported in the English literature. We present a posterior intratracheal RMS in a 6-year-old male with previous Neuraxis radiation with tracheostomy and emphasize the utility of pathological evaluation of tracheal mucosal granulation in patients with increased risk factors for head and neck malignancies.

 Case Report



A 6-year-old tracheostomized male presented to the Pediatric Aerodigestive Clinic at a tertiary care hospital for routine follow-up. The child had a resection of a primitive neuroectodermal tumor of the brainstem 3.5 years previously, followed by postoperative chemotherapy and soon thereafter radiation, both 3 years prior. Chemotherapy comprised six cycles of vincristine, cyclophosphamide, etoposide, and carboplatin. Three weeks after treatment, the patient developed respiratory failure eventually requiring a permanent tracheostomy for respiratory support. At subsequent follow-up 3 years later, an awake in-office fiber-optic evaluation through the tracheostomy tube revealed a fleshy mass obstructing the distal end of the tracheostomy, suspicious of granulation tissue. The child underwent direct laryngoscopy and bronchoscopy, which confirmed the presence of a mucosalized, exophytic mass arising from the posterior tracheal wall approximately 3 cm proximal to the carina [Figure 1]. The lesion was excised using a flexible CO2 laser (Omniguide, Inc., Cambridge, MA, USA) in pulsed mode set at a power of 5 W. The laser fiber was attached to a 4-mm, 30-cm optical telescope, and the excision was achieved while having the child breathe spontaneously during the procedure. Due to the suspicious location of the lesion on the posterior tracheal wall, a biopsy was obtained.{Figure 1}

The lesion was hypercellular and included elongated strap cells with eosinophilic cytoplasm, characteristic of rhabdomyoblasts [Figure 2]. Desmin was positive, and occasional nuclei stained with myogenin, a pattern seen with ERMS [Figure 3]. The child underwent a chest computed tomography scan to define the extent of the lesion, which revealed no lesions in the trachea or in the mediastinum. Subsequent flexible indirect laryngoscopy showed a 1.5 cm × 1.5 cm indurated area in the posterolateral endotracheal wall distal to the tracheostomy tube. After evaluation by pediatric oncology, the patient was staged as clinical Group 1, Stage 3 ERMS. The patient subsequently underwent treatment with dactinomycin and cyclophosphamide. A rigid bronchoscopy 1 year after presentation revealed no evidence of tumor. The child is alive with any evidence of recurrence at 2-year follow-up.{Figure 2}{Figure 3}

 Discussion



The development of granulation tissue is a common occurrence in pediatric tracheostomies. It is most commonly encountered around the tracheostomy stoma and along the mucosa of the tracheal wall, particularly the anterior or lateral walls near the end of the tracheostomy tube. Yaremchuk reported granulation tissue development in up to 10%–80% of tracheostomized patients, with a decreased frequency of surgery required to treat granulation tissue in patients who underwent more frequent tracheostomy changes.[2] Studies from animal models have shown that ionizing radiation is trigger for the development of RMS, in addition to other chemical (heavy metals, polycyclic aromatic hydrocarbons) and biologic (viral proteins and p53 or growth factor alterations) agents.[1],[3] While the majority of RMSs are believed to develop sporadically, there is also an association between RMS and certain genetic syndromes, such as Li-Fraumeni syndrome, Costello syndrome, Noonan syndrome, Von Recklinghausen disease, Rubinstein–Taybi syndrome, hereditary retinoblastoma, Gorlin basal cell carcinoma nevus syndrome, and Beckwith-Wiedemann syndrome.[2]

Given the high frequency of granulation tissue and the rarity of the development of RMS of the tracheal or trachealis muscle, there may be a tendency among otolaryngologists to fail to consider the possibility of malignancy when performing bronchoscopy and laser ablation of granulation tissue. In our case, the high-risk history of the patient involving ionizing radiation, as well as the uncommon location (posterior tracheal wall) of the granuloma, prompted a biopsy, which revealed the RMS.

We conducted a PubMed and EMBASE search for all reported cases of tracheal RMS, using the terms “Pediatric” or “Tracheal” and “Sarcoma” or “Rhabdomyosarcoma.” This resulted in four previously reported cases [Table 1] but none in a previously tracheostomized child (three patients underwent tracheostomy at or after discovery of the RMS[4],[5],[6],[7]). All except the present cases were primary RMSs in children with no previous history of malignancies. The most common presenting symptom in these cases was new-onset stridor and/or dyspnea as would be expected from intratracheal pathology. All the reported cases, including the present one, were embryonal with a mean age of 8.2 years. As with RMS arising at other locations, ERMS is the most common histological variant. However, what differentiates our case is the development of the lesion in a previously tracheostomized child, biasing our initial clinical diagnosis toward tracheal granulation tissue. An analysis of these cases, including the present case, reveals that tumors limited to the trachea usually respond well to surgical excision followed by multimodality chemotherapy and radiation. The Intergroup Rhabdomyosarcoma Study IV advocated the combined use of vincristine, actinomycin D, and cyclophosphamide, plus or minus radiotherapy.[8] Surgical excision can be performed by debulking the tumor with cold instruments or with use of the laser. Considering the usual distal location of the lesion, flexible CO2 laser provides precise excision while minimizing bleeding, thereby improving visualization. The delivery of the flexible fiber can be done through the side port of the bronchoscope, or, as in our case, it can be attached to the rigid telescope to provide better control and visualization of the lesion.{Table 1}

 Conclusion



Even though tracheal granulation is common in tracheostomized patients, a histopathological examination of excised “granulation tissue,” particularly granulomas emanating from the posterior wall, should be considered in patients with an increased risk for tumor development, such as patients with a history of head and neck radiation.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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