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LETTERS TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 4  |  Page : 818-821

Turban-like transcalvarial destructive mass in a young child, with extensive extracranial and intracerebral involvement: A cytological diagnosis and review


1 Department of Pathology, G B Pant Institute of Post Graduate Medical Education and Research, New Delhi, India
2 Department of Neurosurgery, G B Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Date of Web Publication5-Jul-2016

Correspondence Address:
Kaushik Majumdar
Department of Pathology, G B Pant Institute of Post Graduate Medical Education and Research, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.185358

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How to cite this article:
Gupta RK, Majumdar K, Jagetia A, Saran RK. Turban-like transcalvarial destructive mass in a young child, with extensive extracranial and intracerebral involvement: A cytological diagnosis and review. Neurol India 2016;64:818-21

How to cite this URL:
Gupta RK, Majumdar K, Jagetia A, Saran RK. Turban-like transcalvarial destructive mass in a young child, with extensive extracranial and intracerebral involvement: A cytological diagnosis and review. Neurol India [serial online] 2016 [cited 2020 Oct 22];64:818-21. Available from: https://www.neurologyindia.com/text.asp?2016/64/4/818/185358


Sir,

Primary Ewing's sarcoma (ES) of the cranial bone is uncommon and accounts for <1% of all ES.[1] None of the published literature has highlighted the role of fine needle aspiration (FNA) and immunocytochemistry (ICC) on cell block in the diagnosis of primary calvarial ES.[2] Herein, we describe a large parietal bone ES with calvarial destruction and extensive extracranial, intracerebral, and intraorbital involvement in a young boy, which was diagnosed by FNA and ICC. Cytodiagnosis can be an important tool when invasive biopsy, surgical resection, and histological examination are not possible for the highly aggressive, large, friable, vascular mass lesions.

A 12-year-old male child was referred to the Neurosurgery Department with the chief complaints of swelling over the cranium and headache since 2 months and discharge from the wound since 5 days. The child was apparently well 2 months back when he had a head injury, following which he complained of headache and the development of progressively increasing marble-like swelling over the cranium. A local doctor tried an incision and drainage. Due to further increase in the size of the swelling with discharging serosanguinous fluid from the sutured area, he was referred to our hospital. On examination, a huge swelling in the parietal region measuring approximately 15 cm diameter was noted [Figure 1]a.
Figure 1: Photographs of the child showing (a) a large turban-like parietal region mass with right eye proptosis and subconjunctival hemorrhage; (b) at 3-month follow-up, showing appreciable reduction in the tumor size

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Contrast enhanced computed tomography scan revealed a large lobulated transcalvarial mass measuring 10 × 9 × 8.7 cm across the right parietal bone extending into the subgaleal scalp soft tissue and encasing the superior sagittal sinus. The lesion caused destruction of the parietal bone with alteration of the intradiploic signal intensity. The mass was infiltrating into the underlying brain parenchyma with marked compression and displacement of the frontoparietal cortex and the presence of midline shift [Figure 2]a. Intense contrast enhancement, with calcification and necrosis were also seen [Figure 2]b. A radiological differential diagnosis of ES or anaplastic meningioma was offered. FNA was performed for rapid confirmation of the diagnosis before the initiation of treatment. Digital subtraction angiography and endovascular tumor embolization were attempted. Multiple feeders from the right internal maxillary artery with a tumor blush were seen [Figure 2]c and [Figure 2]d. However, embolization was not feasible due to difficulty in cannulation of the tortuous vessel that also led to vasospasm of the blood vessels. The child further deteriorated and developed right eye proptosis, subconjunctival haemorrhage, and diminution of vision likely due to the intraorbital extension of the tumor. Chemotherapy (cyclophosphamide 1200 mg, adriamycin 80 mg, vincristine 300 mg, and mesna 300 mg) was started following which the swelling reduced substantially; however, the child developed severe pancytopenia. Chemotherapy was, thus, discontinued and he was transfused with 2 units of packed cells, 6 units of random donor platelets, and granulocyte colony stimulating factor (150 µg). After improvement of the blood counts, he was given 2 more cycles of chemotherapy. At present, the patient has also received 3 fractions of radiotherapy; and subsequent treatment is scheduled. At 3 months of follow-up, he showed clinical improvement in the form of reduction of the tumor size and a feeling of well-being [Figure 1]b.
Figure 2: (a) Contrast enhanced computed tomography scan showing a large lobulated transcalvarial mass across the right parietal bone (arrow) causing bony destruction of the parietal bone and infiltrating into the underlying brain parenchyma with marked compression and displacement of the frontoparietal cortex; (b) the mass is showing intense postcontrast enhancement (arrow), with calcification and necrosis; (c and d) digital subtraction angiography showing multiple feeder vessels within the mass (arrow)

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The FNA cytology smears were stained with May–Grunwald–Giemsa and hematoxylin and eosin, and a cell block was prepared. The smears were richly cellular and showed sheets of round-to-oval monomorphic cells with hyperchromatic nuclei, nuclear moulding, inconspicuous nucleoli, and scanty amount of cytoplasm [Figure 3]a. However, tadpole cells, described by Klijanienko et al.,[3] in 10% of peripheral ES, were not observed. Occasional periodic acid Schiff positive diastase sensitive cytoplasmic granules were also noted. Frequent mitotic activity was noted. The cell block also showed a similar small blue round cell tumor with cells arranged in solid sheets and large groups [Figure 3]b. ICC performed on the cell block was focally positive for synapatophysin (Thermo Fisher Scientific, USA) [Figure 3]c and [Figure 3]d and CD99 (Dako, Denmark), whereas S-100 (Thermo Fisher Scientific, USA) and epithelial membrane antigen (Thermo Fisher Scientific, USA) were negative [Figure 3]c.
Figure 3: Photomicrographs showing cytomorphology and immunocytochemistry features (a) The smear is richly cellular and shows a tumor comprising mainly of round-to-oval monomorphic cells with hyperchromatic nuclei, inconspicuous nucleolei, and scant cytoplasm. Arrow showing a mitotic figure (MGG × 400); (b) the cell block showing a similar small monomorphic round cell tumor arranged in sheets, (c) immunocytochemistry on cell block showing diffuse membranous CD99 positivity [×400] (arrow), and (d) cytoplasmic synaptophysin positivity [×400] (arrow)

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Calvarial ES are extremely rare; a single case series and only few reports have been described in the English literature.[4] This tumor usually presents as a rapidly growing, large fungating mass developing over weeks to months. Additional symptoms such as headache, neurological deficits, and diminution of vision may occur due to its intracranial and intraorbital extension. The child mentioned here also had severe headache, subconjunctival hemorrhage, proptosis, and marked diminution of vision. Sometimes, a calvarial ES presents with signs of markedly raised intracranial pressure, necessitating an emergent craniectomy.[5]

Calvarial ES behaves differently in comparison to its peripheral counterparts. These lesions have a much lower incidence of metastases; however, their invasive tendency may preclude gross total resection, require an aggressive multimodality treatment, with often an unfavorable outcome. The mesenchymal stem cell has been hypothesized as the cell of origin; however, other types of cells may also be involved, which explains its ability to occur at multiple sites.[6] Among cranial ES, calvarial and meningeal locations have a better outcome in comparison to skull base, which is likely due to its accessibility and the amount of resection possible.[4]

Most cases delineate the characteristic t(11; 22) translocation between the EWS and FLI1 genes, which can be demonstrated by molecular techniques. A multidisciplinary approach including surgical excision along with chemotherapy and radiotherapy results in a good outcome with a 2–5 year disease free survival in 50–80% patinets.[7] In this case, because of multiple feeders from the right internal maxillary artery, endovascular tumor embolization was attempted but without success; therefore, chemotherapy was initiated instead of surgical resection. The outcome is usually good if there is no early recurrence. Early recurrence, presence of metastasis, and extremes of age are probably responsible for a poor prognosis. The earlier literature has not emphasized the importance of FNA in the management of calvarial ES. Although Ahmed et al.,[8] have mentioned FNA cytology as a diagnostic modality in their report, the cytological features or ICC were not described. Synaptophysin and CD99 expression by ICC in this case confirmed the diagnosis, guided the management options, and also indicated its relationship with primitive neuroectodermal tumors.

To conclude, FNA is a rapid, accurate and minimally invasive procedure for a calvarial ES in combination with cell block and ICC. It can provide a definite diagnosis and guide the therapeutic options, especially in large, highly aggressive, infiltrating vascular tumors, wherein surgery may not be feasible.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN. Primary Ewing's sarcoma of the cranium. Neurosurgery 2000;46:62-8.  Back to cited text no. 1
    
2.
Gautam U, Srinivasan R, Rajwanshi A, Bansal D, Marwaha RK, Vasishtha RK. Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology. Am J Clin Pathol 2010;133:633-45.  Back to cited text no. 2
    
3.
Klijanienko J, Couturier J, Bourdeaut F, Fréneaux P, Ballet S, Brisse H, et al. Fine-needle aspiration as a diagnostic technique in 50 cases of primary Ewing sarcoma/peripheral neuroectodermal tumor. Institut Curie's experience. Diagn Cytopathol 2012;40:19-25.  Back to cited text no. 3
    
4.
Krishnamani K, Kumar TN, Gandhi LV, Raghunadharao D, Sadashivudu G, Megha U. Primary Ewing's sarcoma of the cranium: Case series and review of literature. J Cancer Res Ther 2014;10:377-80.  Back to cited text no. 4
    
5.
Tournut P, Turjman F, Laharotte JC, Froment JC, Gharbi S, Duquesnel J. Primary Ewing's sarcoma of the skull presenting as an acute surgical emergency. Child's Nerv Syst 1994;10:193-4.  Back to cited text no. 5
    
6.
Salunke PS, Gupta K, Pfeifer JD. The cell of origin of cranial Ewings sarcoma: The dilemma persists. Acta Neurochir 2012;154:567-8.  Back to cited text no. 6
    
7.
Vohra VG. Roentgen manifestations in Ewing's sarcoma. A study of 156 cases. Cancer 1967;20:727-33.  Back to cited text no. 7
    
8.
Ahmad FU, Suri A, Mahapatra AK, Ralte A, Sarkar C, Garg A. Giant calvarial Ewing's sarcoma. Pediatr Neurosurg 2004;40:44-6.  Back to cited text no. 8
    


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