Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 17  |  Issue : 1  |  Page : 37-41

Primary orbital rhabdomyosarcoma in an 11-year-old boy: A management challenge in a resource limited environment


1 Department of Ophthalmology, Benue State University Teaching Hospital, Makurdi, Benue State, Nigeria
2 Department of Pathology , Benue State University Teaching Hospital, Makurdi, Benue State, Nigeria
3 Department of Radiology, Benue State University Teaching Hospital, Makurdi, Benue State, Nigeria

Date of Web Publication7-Jan-2015

Correspondence Address:
Keziah N Malu
Department of Ophthalmology, Benue State University Teaching Hospital, PMB 102131, Makurdi, Benue State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2276-7096.148696

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  Abstract 

Rhabdomyosarcoma (RMS) is a very rare cancer, with only about 250-350 cases of children below 21 years of age seen in USA every year. Nigerian hospital studies have shown about 1-4 cases a year. Primary orbital RMS occurs mainly in children, with 90% below the age of 16 and a mean age of 5-7 years. It arises from the orbit and eyelid in up to 10% of all cases. The superonasal part of the orbit is the most favored position, giving rise to proptosis with lateral displacement of the eyeball. Orbital RMS enjoys a relatively good prognosis as a result of improved radiological imaging with computed tomography and magnetic resonance imaging, and adequate chemotherapy or radiation in advanced nations. In developing societies with limited resources, it still carries a poor prognosis. We present a case report of an 11-year-old boy with rapidly progressing tumor, which was confirmed histologically to be orbital RMS. The case report illustrates challenges faced in the management of orbital RMS in a resource limited environment.

Keywords: Diagnostic challenges, primary orbital rhabdomyosarcoma, therapeutic difficulties, resource limited environment


How to cite this article:
Malu KN, Ngbea JA, Mohammand H. Primary orbital rhabdomyosarcoma in an 11-year-old boy: A management challenge in a resource limited environment. J Med Trop 2015;17:37-41

How to cite this URL:
Malu KN, Ngbea JA, Mohammand H. Primary orbital rhabdomyosarcoma in an 11-year-old boy: A management challenge in a resource limited environment. J Med Trop [serial online] 2015 [cited 2019 Oct 24];17:37-41. Available from: http://www.jmedtropics.org/text.asp?2015/17/1/37/148696


  Introduction Top


Rhabdomyosarcoma (RMS) is a very rare cancer, with only about 250-350 cases of children below 21 years of age seen in USA every year. [1],[2],[3] Nigerian hospital studies have shown about 1-4 cases a year. [4],[5],[6] Primary Orbital RMS occurs mainly in children below the age of 16 years in about 90%, with a mean age of 5-7 years. It is the most common sarcoma of childhood ocular tumors and is more common in boys. [2],[3]

RMS is derived from undifferentiated mesenchymal cell rests with potential of differentiating into striated muscle.

About 40% of new cases of RMS arise from the head and neck, including parameningeal sites.

The orbit and eyelid make up 10% of all new cases. Most ocular RMS arises from the soft tissues of the orbit and on some occasions can arise in other ocular adnexal structures and even within the eye. [3] The superonasal part of the orbit is the most favored position, giving rise to proptosis with lateral displacement of the eye ball. [3],[7] Tumors arising from the orbit and nonparameningeal sites such as testis and female genital tract are considered as carrying favorable prognosis.

One in 10 children with RMS are diagnosed with other syndromes such as neurofibromatosis, Beckwith-Wiedemann, the Li-Fraumeni, and Costello syndromes. [8]

Orbital RMS enjoys a relatively good prognosis in advanced nations as a result of improved radiological imaging with computed tomography (CT) and magnetic resonance imaging (MRI), and adequate chemotherapy or radiation.

In developing societies with limited resources, it still carries a poor prognosis. This is as a result of limited facilities available and financial capacity of those suffering from the disease.

This case report illustrates challenges faced in the management of a child with orbital RMS in a resource limited environment.


  Case Report Top


An 11-year-old boy presented with a history of painless progressive protrusion of the left eye (LE) for 4 weeks. The eye protrusion was noticed by the mother while giving the boy a bath. He denied history of trauma. There was neither a history of epistasis nor nose block. He developed normally like the rest of his siblings. He had gone to a private hospital where some eye drops were given, but without relief.

His visual acuity was right eye (RE) 6/36, (there was no visual improvement with pin hole), LE counting fingers (CF). Other findings in the RE were essentially normal including a canthal-corneal measurement of 18 mm with a plastic ruler. There was LE proptosis of 35 mm with diffuse orbital mass pushing the eye slightly laterally, and mild conjunctival chemosis. The cornea was clear and pupil reacted briskly to light. Except for the cup/disc ratio of 0.4 RE and 0.5 LE the fundi were normal, especially with no signs of chorioretinal folds. An impression of an orbital mass with differentials of idiopathic orbital inflammatory disease and orbital cellulitis were made. Laboratory investigations showed an erythrocyte sedimentation rate of 70 mm/h westergreen, white blood cells 5.6 × 10 9 , neutrophils 44%, lymphocytes 51%, monocyte 0.2% and eosinophils 0.3%, presence of anisocytosis, hypochromatosis, and adequate platelets. The retroviral screening was nonreactive. Skull and orbital X-ray, CT scan and MRI were requested, but only the skull X-ray was carried out. The orbital radiographs showed the presence of an expansion, sclerosis and thickening of the medial wall of the left orbit with overlying soft tissue swelling with a diagnosis of an expansile left orbital tumor, with differentials of idiopathic orbital inflammatory disease and lympho-proliferative lesion.

He was initially placed on prednisolone (Kunimed Pharmachem Ltd.) 10 mg 3 times daily and guttae dexamethasone (Drugfield Pharmaceutical Ltd.) 3 times daily for 1 week. The visual acuity improved to RE 6/18, but the LE was still CF with increased proptosis (RE 20 mm and LE 40 mm) [Figure 1].
Figure 1: An 11-year-old boy with left eye orbital tumor (rhabdomyosarcoma) with lateral displacement of the eye

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The patient was referred to an oncologist, but unfortunately he did not go for the consultation, choosing rather to seek traditional treatment.

The patient was seen 2 weeks later. He had developed severe pain in the LE, had a huge retro-orbital mass with proptosis and prolapse of the eye, severe chemosis and corneal excoriation. The visual acuity LE was nonperception of light, and he had maxillary swelling [Figure 2].
Figure 2: The patient 2 weeks after presentation. Note gross prolapse of left orbital tumor, excoriation of the cornea, and swollen maxillary region

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The patient complained of generalized body weakness, had left submandibular lymphadenopathy, but there were no other palpable masses. An impression of Burkitts lymphoma was made.

The urea and electrolytes and liver function tests were within normal range. Repeat blood counts showed packed cell volume of 36.4%, target cells 2+, teardrop poikilocytes 1+, marked neutrophil leukocytosis, and markedly elevated platelets (557/mm 3 ).

The patient was started on cefuroxime (by Aunbindo Pharma Ltd.) 375 mg 12 hourly, metronidazole (by May and Baker Nigeria Plc.) 200 mg 8 hourly and 5% dextrose 500 ml alternating with 0.9% normal saline 500 ml 8 hourly before surgery and he was prepared for exenteration of the LE (because of the bulky painful tumor). The cefuroxime and metronidazole were continued for 5 days postoperatively, whereas the dextrose and saline were stopped after 48 h. At surgery a firm cheesy yellow-whitish orbital tumor of more than 5 cm was found. The tumor did not arise from extra-ocular muscles [Figure 3].
Figure 3: The orbital tumor has pushed the eye out of the socket, and the eye is seen sitting on top but separate from the tumor

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It was excised piece meal as there was no space between the tumor and the orbital bones. The orbital bones were spared. The tumor mass was excised, but the submandibular lymph nodes were not removed. [Figure 4] shows empty socket after removal of the tumor (modified exenteration).
Figure 4: The left orbit after the removal of the orbital tumor (modified exentration)

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Postoperatively, he was transfused two units of O-positive whole blood.

The histology confirmed embryonic RMS [Figure 5] and [Figure 6].
Figure 5: Histologic section of the exercised left orbital mass showing malignant rhabdomyoblasts in a loose fibromyxoid stroma, these cells are round, oval, spindled with prominent eosinophilic cytoplasm. Hyperchromatic, atypical nuclei is also observed (H and E, ×20)

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Figure 6: Histologic section of the orbital mass showing tadpole cells (rhabdomyoblasts) (H and E, ×40)

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Pediatric oncologist was consulted to co-manage the patient.

On the 3 rd postoperative day, however the patient lost vision in the fellow eye (RE). Visual acuity was perception of light with afferent pupillary reflex defect, but no lesion was seen on fundoscopy.

A CT scan of the skull and orbit was requested since MRI was not available at the hospital and (the patient had no finances to travel to the nearest center with the facility, which was 330 km away) the CT scan was also malfunctioning that week and therefore the investigation could not be carried out.

Systemic steroids were added to his medications (prednisolone [by Kunimed Pharmachem Ltd.] 10 mg tds for 2 weeks). The visual acuity improved only to hand movement.

The socket was healing well [Figure 7].
Figure 7: The patient 3 weeks post exentration of the left eye. Note the dilated right eye pupil

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The Oncologist reviewed and offered to start him on chemotherapy, but the patient's relatives requested discharge against medical advice because of lack of funds. They claimed they were taking him home for prayers. The social welfare was contacted to help, but the relatives refused claiming they had other young children at home to cater for.


  Discussion Top


This was a case of RMS with management challenges. Ocular RMS can pose diagnostic and management challenges even to an ocular oncologist because it does mimic other ocular neoplasms such as neuroblastoma, chloroma, lymphangioma, Burkitts lymphoma, and orbital cellulitis or nonspecific inflammatory orbital conditions. [7]

In the present case, it took 1 week to get the X-ray and the patient was not able to do the CT scan for financial reasons. This highlights the challenges faced in the management of patients with cancers in the developing world. Poverty is a major challenge. It took the parents 1 week to source for finances for an X-ray and of course CT scan were beyond their means. When later money was made available through some aid the equipment had broken down.

In our environment where there are hardly any biomedical engineers capable of handling repairs of this equipment, it becomes quite a frustrating issue for physicians managing patients. Some of these challenges have been highlighted in other studies in the country. [9],[10]

Primary orbital RMS is said to affect mainly young children and 90% occur before the age of 16 years with a mean age of 5-7 years. Males are slightly more affected than females, with male to female ratio of 5:3. [1],[2],[3] Our patient was an 11-year-old boy.

About 40% of new cases of RMS arise from the head and neck, including parameningeal sites. Mandong and Ngbea [4] in a 10-year review of 35 histopathological confirmed cases of RMS in a tertiary hospital in Jos, Nigeria found 21 (60%) of them were of head and neck region, out of which 3 (8.6%) were of the orbit. Most ocular RMS occur in the soft tissues of the orbit and on some occasions can arise in other ocular adnexal structures and even within the eye. [3] The superonasal part of the orbit is the most favored position giving rise to proptosis with lateral displacement of the eye [7] as was the case with this patient; the tumor was not attached to the extra-ocular muscles at all.

Tumors arising from the orbit and non-parameningeal sites such as testis and female genital tract are considered as carrying favorable prognosis. The tumor response to chemotherapy and radiation is good, and a maximum tumor excision before adjunctive chemotherapy and radiation may improve survival benefit. [11],[12],[13] This would have benefited our patient if he was able to have an adjunctive chemotherapy and radiation. However, the relatives opted for alternative nonmedical therapy because of lack of funds. In our society when parents are told a child has cancer they would rather go home than "waste" their scare resources on what they believe as a useless venture. Many believe that cancer is not curable so why "waste" money on what cannot be remedied. Secondly, there is so much belief in traditional medicine. Patients are told by the traditional healer that the hospital is the last place they should consult. As a result, patients go back and forth between orthodox and traditional remedies as was the case in our patient.

One in 10 children with RMS is diagnosed with other syndromes with known genetic mutations.

Orbital RMS may also occur as a secondary tumor post radiation treatment for retinoblastoma and squamous cell carcinoma of the eyelid. [3] Most do not have any detectable genetic mutation at all. Our patient was a normal child with no identifiable associated syndrome, and no abdominal masses. He had no family history of cancers or any of the associated syndromes above. He had normal growth with normal mile stones like the rest of the children in the family. However, gene studies were not carried out because facilities were not available.

There are four main histopathologic types of RMS; the embryonal, alveolar, pleomorphic, and botyroid. The most common type of orbital RMS seen is the embryonal, and it carries a favorable prognosis. [3],[7] The histopathology of our patient's tumor was also embryonal in origin.

Primary orbital RMS characteristically presents with a rapid onset of unilateral proptosis and displacement of the globe either inferiorly or inferotemporally and is painless at the initial stage. On few occasions the proptosis is gradual. In the case of this patient, the total presentation was within 2-3 months. Our patient did not have pain at the initial stage and hence it looked like an idiopathic inflammatory disorder (orbital pseudotumor).

Paranasal sinus RMS invading the orbit may present like primary orbital RMS, but with nasal or sinus congestion and epistaxis and rarely, nasopharyngeal RMS can invade the orbital apices leading to rapid visual loss secondary to the optic nerve compression.

In this patient, CT scan and MRI could not be done, so it was difficult to tell the cause of the loss of vision in the remaining RE. He did not present with epistaxis and with no symptoms of nose block. Radiological imaging is important in both diagnosis and management and follow-up of patients with orbital RMS. The CT scan usually shows a well-circumscribed non-calcified homogenous mass which appears isodense to the extra-ocular muscles. The mass enhances following the administration of contrast. Bone erosion and invasion of the paranasal sinuses or nasopharynx occurs in advance disease. MRI, T1-weighted will also show a mass of intermediate intensity, or isointense to muscles. T2-weighted images are usually hyperintense. The mass typically enhances with gadolinium. [14]

None of these imaging modalities could be carried out on this patient for logistic reasons.

The treatment of orbital RMS involves a combination of surgery, chemotherapy, and irradiation. In more developed and industrialized societies with well-equipped centers for treating cancers a 5-year survival rate of 90-95% for patients with RMS is common. In places where only surgical excision and chemotherapy are available, the survival rate is low, with 3-year survival of 30-40%. [11],[12],[13] Our patient could not even undergo chemotherapy ostensibly for financial reasons, probably compounded by the belief that it was a losing battle.

Children and adolescence with cancers are managed by multidisciplinary team of primary care physician, radiation oncologist, pediatric oncologist/hematologist, pediatric surgical subspecialists, pediatric nurse specialists, social workers, rehabilitation specialists for optimal benefit of the child. In our society such teams do not exist, leading to the challenges of managing such cases by an ophthalmologist who may not even be an ocular oncologist.

Our patient had a favorable chance of at least 3 or more year survival if the chemotherapy was undertaken and some maximum cooperation with the parents had existed. The socket was healing well. The finances for the chemotherapy, hospital stay and the needs of the family back at home all needed to be taken care of hence their decision to opt out of the hospital management. Some belief pattern might also have informed their decision. They had previously consulted a traditional healer and again were going to take the boy to a prayer house.


  Conclusion/Recommendation Top


This case report illustrates the limitation in making diagnosis and challenges of management of an 11-year-old boy with orbital RMS in a resource limited environment.

There is a need for Government to aid cancer patients so as to strengthen compliance and improve outcome, just as it does for patients with HIV and tuberculosis.


  Acknowledgements Top


We are most grateful to our patient who because of poverty, ignorance and limited resources was not able to benefit maximally from the modern management of his cancer condition.

 
  References Top

1.
Wharam M, Beltangady M, Hays D, Heyn R, Ragab A, Soule E, et al. Localized orbital rhabdomyosarcoma. An interim report of the Intergroup Rhabdomyosarcoma Study Committee. Ophthalmology 1987;94:251-4.  Back to cited text no. 1
    
2.
Gandhi PD, Fleming JC, Haik BG, Wilson MW. Ophthalmic complications following treatment of paranasal sinus rhabdomyosarcoma in comparison to orbital disease. Ophthal Plast Reconstr Surg 2011;27:241-6.  Back to cited text no. 2
    
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Shields JA, Shields CL. Rhabdomyosarcoma: Review for the ophthalmologist. Surv Ophthalmol 2003;48:39-57.  Back to cited text no. 3
    
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Mandong BM, Ngbea JA. Childhood rhabdomyosarcoma: A review of 35 cases and literature. Niger J Med 2011;20:466-9.  Back to cited text no. 4
    
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Fasina O. Pattern of presentation and outcome of ophthalmic rhabdomyosarcoma in Ibadan. Afr J Med Med Sci 2013;42:165-9.  Back to cited text no. 5
    
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Umar AB, Ochicha O, Iliyasu Y. A pathologic review of ophthalmic tumors in Kano, Northern Nigeria. Niger J Basic Clin Sci 2012;9:23-6.  Back to cited text no. 6
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Wurm J, Constantinidis J, Grabenbauer GG, Iro H. Rhabdomyosarcomas of the nose and paranasal sinuses: Treatment results in 15 cases. Otolaryngol Head Neck Surg 2005;133:42-50.  Back to cited text no. 11
    
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Sutow WW, Lindberg RD, Gehan EA, Ragab AH, Raney RB Jr, Ruymann F, et al. 3-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck: Report from the Intergroup Rhabdomyosarcoma Study. Cancer 1982;49:2217-21.  Back to cited text no. 12
    
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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. 13
    
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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