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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 2  |  Issue : 1  |  Page : 9

Botryoid rhabdomyosarcoma of the tarsal conjunctiva in a 9-year-old boy


Departament of Ophthalmology, Pontifical Catholic University of Campinas, Ouro Verde Hospital Complex, Campinas, Brazil

Date of Submission14-Jan-2020
Date of Acceptance26-Feb-2020
Date of Web Publication23-Apr-2020

Correspondence Address:
Dr. Bianca Prado Patrus
Av Imperio do Sol Nascente, 581. Postal Code: 13033050, Campinas - SP
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/PAJO.PAJO_1_20

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  Abstract 


The objective is to report a rare case about rhabdomyosarconma in PUC Campinas Hospital. This was a retrospective case report. A 9-year-old boy which presented a superior tarsal conjunctival lesion of the left eye which had a rapid growth in days. The patient underwent a biopsy which revealed an embryonal rhabdomyosarcoma stage III. The patient was submitted chemotherapy according to the Intergroup Rhabdomyosarcoma Study IV protocol and radiotherapy. This is a report discussed about this unusual tumor presentation in the city of Campinas, São Paulo state, Brazil.

Keywords: Conjunctiva rhabdomyosarcoma, conjunctival tumor, ocular oncology


How to cite this article:
Patrus BP, Ferreira HS, Faria DA, Sobrinho MV, Burnier SV, Faria O. Botryoid rhabdomyosarcoma of the tarsal conjunctiva in a 9-year-old boy. Pan Am J Ophthalmol 2020;2:9

How to cite this URL:
Patrus BP, Ferreira HS, Faria DA, Sobrinho MV, Burnier SV, Faria O. Botryoid rhabdomyosarcoma of the tarsal conjunctiva in a 9-year-old boy. Pan Am J Ophthalmol [serial online] 2020 [cited 2020 Nov 25];2:9. Available from: https://www.thepajo.org/text.asp?2020/2/1/9/283174




  Introduction Top


Rhabdomyosarcoma is a tumor composed of primitive myogenic cells. This is the most common primary orbital tumor in children, accounting for 5% of all pediatric tumors. The main tumor sites in decreasing order of incidence are head and neck, trunk and extremities.[1]

The conjunctival localization is uncommon, with few reports in the literature. Histologically the tumor can be classified in: embryonic, alveolar, and pleomorphic, being the embryonic the most common. It manifests with elongated cells and scattered rhabdomyoblasts with eosinophilic cytoplasm. A botryoid is a less common variant of the embryonic tumor, its name is presented by its grape-like presentation and gelatinous and soft consistency.[2]

Cellulitis, connective papilloma, connective lymphoma, lipoma, myxoma, conjunctival intraepithelial neoplasia, and parinaud oculoglandular syndrome are differential diagnoses of this pathology.[3]

The orbital rhabdomyosarcoma is classified by an international staging according to the Intergroup Rhabdomyosarcoma Study (IRS) postsurgical staging system. The Group I: localized disease completely resected by the biopsy, Group II: microscopic disease remaining after biopsy, Group III: gross residual disease remaining after biopsy, and Group IV: distant metastasis present.[4]

The treatment for this tumor is based on surgery, radiotherapy, and chemotherapy that aim to minimize the sequel of late treatment, besides curing the disease.


  Case Report Top


A 9-year-old child, male, previously healthy. Denied previous eye disease and surgery.

The patient had upper eyelid growth in the left eye 2 weeks ago who claimed that happened after sand fell in the same eye [Figure 1]. In the first evaluation, the visual acuity in the right eye was 20/20 and the left eye 20/30. On the examination in the left eye, there was the presence of an upper eyelid mass that bulge the tarsal conjunctiva, leaving it exposed. The motility of the extrinsic musculature was preserved. Slit-lamp examination showed a transparent cornea, formed anterior chamber. No fluorescein uptake in the cornea and no seidel. Tonometry: 12 mmHg. Fundoscopy: Applied retina, preserved macula and physiological cupping of the optic nerve. The right eye was normal.
Figure 1: First inspection of patient's eye

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Blood count, C-reactive protein, and erythrocyte sedimentation rate were unchanged.

Computed tomography (CT) scan showed edema in the upper eyelid region in the left eye and no orbital infiltration. In the 1st week, it was the patient use of moxifloxacin hydrochloride and dexamethasone disodium phosphate topical in each 6 h. The patient was evaluated two times during the 1st week. In the 2nd week, the patient reported increased lesion. On inspection, the eyelid had doubled in size from the first assessment and did not allow for the assessment of the eyeball [Figure 2].
Figure 2: Patient's eye inspection 9 days after the first assessment

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The patient was followed daily at the eye plastic outpatient clinic. After 1 week, the patient underwent lesion excision for biopsy. There were no complications in the surgery. After the procedure, he used dexamethasone associated with tobramycin for 15 days. The immunohistochemical study revealed the expression for desmin, MyoD1, and myogenin which together with the pathology revealed findings compatible with botryoid embryonic rhabdomyosarcoma [Figure 3]. The patient was referred to an oncological hospital for chemotherapy according to the IRS IV protocol and radiotherapy.[5]
Figure 3: Histopathology and immunohistochemistry of conjunctival rhabdomyosarcoma

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After 2 months of chemotherapy, the patient returned to new evaluation. The visual acuity in the right eye was 20/20 and the left eye 20/40. On the examination in the left eye, there was severe ptosis [Figure 4]. The motility of the extrinsic musculature was preserved. Slit-lamp examination showed a transparent cornea, the anterior chamber formed. No fluorescein uptake in the cornea and Seidel. Tonometry: 12/12 mmHg. On eyelid eversion, there was not a superior tarsal plate. Fundoscopy: Applied retina, preserved macula, and physiological excavation. The right eye was normal. Moreover, the patient continued the treatment.
Figure 4: Inspection of patient's eye after 2 months of treatment

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  Discussion Top


Rhabdomyosarcoma is the most common primary orbital tumor in the pediatric population. The main signs of this pathology are proptosis, eyelid swelling, and blepharoptosis.[6]

In cases with presence of expansive lesion, a tumor hypothesis should always be considered. The patient should be evaluated as soon as possible to program the lesion biopsy since in this situation, the diagnosis is concluded by the pathology, and the treatment depends on the result. Besides, the site must be investigated to define the extension of the lesion by an orbital magnetic resonance imaging scan or a CT scan.

Rhabdomyosarcoma is a life-threatening condition. Among the rhabdomyosarcoma subtypes, the embryonic botryoid has a good prognosis and excellent response to treatment. In this sense, recognition, quickly care, and therapeutic programing are essential for the patient's prognosis.

This case is important because there are few reports on primary conjunctival botryoid rhabdomyosarcoma.

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.



 
  References Top

1.
Essafi H, Hajji I, Aboutoufail S, Ahammou H, Soltani L, Moutaouakil A. A rare location of palpebral rhabdomyosarcoma. Lancet Oncol 2018;19:e65.  Back to cited text no. 1
    
2.
Polito E, Pichierri P, Loffredo A, Lasorella G. A case of primary botryoid conjunctival rhabdomyosarcoma. Graefes Arch Clin Exp Ophthalmol 2006;244:517-9.  Back to cited text no. 2
    
3.
Brichard B, De Potter P, Godfraind C, Vermylen C. Embryonal rhabdomyosarcoma presenting as conjunctival tumor. J Pediatr Hematol Oncol 2003;25:651-2.  Back to cited text no. 3
    
4.
Jurdy L, Merks JH, Pieters BR, Mourits MP, Kloos RJ, Strackee SD, et al. Orbital rhabdomyosarcomas: A review. Saudi J Ophthalmol 2013;27:167-75.  Back to cited text no. 4
    
5.
Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, et al. Intergroup rhabdomyosarcoma study-IV: Results for patients with nonmetastatic disease. J Clin Oncol 2001;19:3091-102.  Back to cited text no. 5
    
6.
Shields CL, Shields JA, Honavar SG, Demirci H. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology 2001;108:2284-92.  Back to cited text no. 6
    


    Figures

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



 

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