Primary orbital neuroblastoma with intraocular extension

Vallinayagam, Muthukrishnan; Rao, Vasudev Anand; Pandian, Datta Gulnar; Ganesan, Niruban

doi:10.4103/0301-4738.169784

Cited by 8 publications

(8 citation statements)

References 10 publications

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“…Ophthalmic involvement occurs in due course of NB in 50% of cases [ 6 ]. However, the initial presentation of this tumor with ophthalmic manifestation is rare and few cases were described in the literature as case reports [ 7 - 8 ]. Its ocular manifestations include orbital mass causing proptosis, ecchymosis, Horner’s syndrome, papilledema, nystagmus, extraocular muscle palsy, ptosis, and retinal striae [ 2 - 3 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Belgaumi et al suggested high dose multi-agent chemotherapy in infants and children under the age of two for prevention of blindness as such groups have the best chance for resolution [ 13 ]. Vallinayagam et al also reported a case of a rapidly progressive primary orbital NB in a two-year-old patient with an excellent response after completion of three cycles of chemotherapy [ 8 ]. Lastly, the prognosis of NB in children is dependent on many factors such as age at diagnosis, disease stage, and histological grade with the highest survival rate found in children under the age of two.…”

Section: Discussionmentioning

confidence: 99%

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Rapidly Progressive Ocular Proptosis as the First Sign of Neuroblastoma in a 16-Month-Old Child: Case Report and Review of Literature

Mandura¹

2022

Cureus

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Neuroblastoma (NB) is a malignant neoplasm accounting for 7.5% of malignancy in children. It can originate anywhere along the sympathetic chain with the adrenal medulla being the most common site in 35% of cases. The initial presentation of orbital metastasis is very unusual that accounts for only 8% of all NB cases. We report a rare case of a 16-month-old girl who initially presented with bilateral rapidly progressive ocular proptosis for two weeks. CT scan of the brain and orbits revealed bilateral heterogeneous lateral orbital lesions, and CT scan of the abdomen and pelvis revealed huge heterogeneous right adrenal lesions. Histopathology of the abdominal mass confirmed the diagnosis of stage IV NB with orbital metastasis and the patient was started on an aggressive chemotherapy regimen. Ophthalmologists have a vital role in the diagnosis of NB which should be considered in the differential diagnosis of any rapidly progressive proptosis in young children. Early investigation and systemic workup should be made immediately, as it is a potentially life-threatening malignant tumor that requires aggressive management.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Rapidly Progressive Ocular Proptosis as the First Sign of Neuroblastoma in a 16-Month-Old Child: Case Report and Review of Literature

Mandura¹

2022

Cureus

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“…In the last 2 malignancies, the consistency of the tumor is firmer than in the case of NBL. 28 The orbit may rarely be the primary locus of the NBL, 26,27,47,61,71 and in contrast to the disease arising from the adrenals, it is resistant to chemotherapy and radiotherapy even at a young age. Hence, extended surgical treatment is necessary, which leads to unilateral blindness.…”

Section: Discussionmentioning

confidence: 99%

“…FIGURE 2. The association between the mean age of the Neuroblastoma patients and ophthalmologic manifestation according to our analysis of 65 case reports and Musarella et al[8][9][10][12][13][14][16][17][18][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37] HS indicates Horner syndrome; OMS, opsoclonus-myoclonus syndrome. *Statistical significance.…”

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confidence: 99%

Ophthalmologic Manifestations of Neuroblastoma: A Systemic Review

Kuchalska

Derwich²,

Gotz‐Więckowska

2022

Journal of Pediatric Hematology/Oncology

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Neuroblastoma (NBL) is the most common extracranial solid tumor found in pediatric patients. It develops from the sympathetic tract tissue. Although the symptoms are associated with tumor localization, sometimes NBL is manifested as ophthalmologic disorders. In this paper, we describe their incidence and the correlation with the prognosis. We searched 2 databases (PubMed and Web of Science) for papers published before April 2022, and concerned pediatric patients with NBL, which caused ophthalmologic changes. We collected 7 papers assessing the occurrence of ophthalmologic findings in the NBL patients, as well as 68 case reports presenting children with orbital changes and NBL, or with other tumors stemming from the sympathetic ganglia. The statistical analysis was performed to synthetize the data. The ophthalmologic signs occurred in 17.89% of the NBL cases; however, they were observed on presentation in 10.68%. The isolated ocular presentation was found in 2.56% of patients, whereas Horner syndrome was most frequent. The ophthalmologic symptoms correlated with patients’ age, primary tumor site, and survival rate. NBL may be challenging to diagnose in cases with isolated ophthalmologic manifestations. Numerous possible ocular signs can be observed, which emphasize the need for multidisciplinary care with regard to the NBL patients.

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“… 22 23 24 Neuroblastoma represents the second most common orbital tumor in children after RMS, and only 8% cases first present with an orbital lesion. 25 Orbital neuroblastoma in children commonly occurs due to metastasis with primary in the abdomen. IHC plays an important role in differentiating neuroblastoma from other small round cell tumors such as retinoblastoma, RMS, and lymphoma.…”

Section: Discussionmentioning

confidence: 99%

Experience with Orbital Tumors from a Tertiary Cancer Centre of North East India: A Pathology Perspective

et al. 2020

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Background The orbit is an anatomically complex structure comprising the globe, extraocular muscles, fat, vascular, nervous, glandular, and connective tissues. A wide variety of neoplasms can arise from different orbital structures, which can create a diagnostic challenge to the pathologists. No formal study has been conducted in this regard in North East India. Aim and Objectives This article aims to document the pattern and prevalence of orbital tumors in our institute and assess the utility of histopathological examination (HPE) and immunohistochemistry (IHC) in the precise diagnosis of these neoplasms. Materials and Methods A retrospective analysis of orbital tumors was performed over a period of 5 years from 2013 to 2018 in the department of pathology at a tertiary cancer center of North East India following all the guidelines of the institutional ethics committee. Results A total of 35 cases of orbital neoplasms, evaluated by HPE and IHC, were found, all of them being malignant tumors. The age range was 4 months to 85 years. Male to female ratio was 1.5:1. The most common tumor found was lymphoma, accounting for 10 cases (28.6%), all of which were non-Hodgkin lymphoma (NHL). All these cases except one occurred in adults, thus making it the most common tumor in adults in this study. Diffuse large B cell lymphoma, not otherwise specified, was the most common NHL, followed by follicular lymphoma, mature T cell NHL, extranodal marginal zone lymphoma, and B cell lymphoblastic lymphoma. Rhabdomyosarcoma and poorly differentiated/undifferentiated carcinoma jointly were the second most common tumors, totaling seven cases (21.21%) each. This was followed by melanoma (three cases), myeloid sarcoma (three cases), Ewing sarcoma/peripheral neuroectodermal tumor (PNET) (three cases), neuroblastoma (one case), and angiosarcoma (one case). Among these, rhabdomyosarcoma, granulocytic sarcoma, Ewing sarcoma/PNET, and neuroblastoma exclusively troubled the children. IHC markers including the lymphoma panel, and soft tissue ones were crucial in the precise diagnosis of the neoplasms encountered. Conclusion A variety of malignant orbital tumors may be seen in clinical practice. Management of these tumors requires a multidisciplinary approach. HPE in conjunction with IHC evaluation is of utmost importance in the veracious recognition of orbital tumors for their proper management.

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Primary orbital neuroblastoma with intraocular extension

Cited by 8 publications

References 10 publications

Rapidly Progressive Ocular Proptosis as the First Sign of Neuroblastoma in a 16-Month-Old Child: Case Report and Review of Literature

Rapidly Progressive Ocular Proptosis as the First Sign of Neuroblastoma in a 16-Month-Old Child: Case Report and Review of Literature

Ophthalmologic Manifestations of Neuroblastoma: A Systemic Review

Experience with Orbital Tumors from a Tertiary Cancer Centre of North East India: A Pathology Perspective

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