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Table of Contents    
LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 2  |  Page : 508-510

Recurrence of Sporadic Intracranial Cerebellar Hemangioblastoma: Case Report and Literature Review


1 Department of Pharmacy Practice (Pharm-D), Indira College of Pharmacy, Pune, Maharashtra, India
2 Consultant, Neurosurgery, Aditya Birla Memorial Hospital, Pune, Maharashtra, India

Date of Submission30-Mar-2020
Date of Decision02-Apr-2020
Date of Acceptance12-Jul-2020
Date of Web Publication24-Apr-2021

Correspondence Address:
Madhavi Ashok Jejurkar
Department of Pharmacy Practice (Pharm-D), Indira College of Pharmacy, Pune - 411033, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.314560

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How to cite this article:
Jejurkar MA, Lagad SG, Ranjan R. Recurrence of Sporadic Intracranial Cerebellar Hemangioblastoma: Case Report and Literature Review. Neurol India 2021;69:508-10

How to cite this URL:
Jejurkar MA, Lagad SG, Ranjan R. Recurrence of Sporadic Intracranial Cerebellar Hemangioblastoma: Case Report and Literature Review. Neurol India [serial online] 2021 [cited 2021 May 15];69:508-10. Available from: https://www.neurologyindia.com/text.asp?2021/69/2/508/314560




Sir,

Microsurgical gross total resection of cerebellar hemangioblastoma is considered the gold standard of treatment with long term risk-free survival. Recurrence in such tumors after gross total resection is extremely rare in sporadic intracranial cerebellar hemangioblastoma (SICH).

Hemangioblastomas are uncommon, highly vascular, and benign tumors that account for 1-2.5% of all intracranial tumors and 3% of all central nervous system tumors (CNS).[1],[2] Complete resection cures sporadic hemangioblastoma but in Von Hippel-Lindau Disease (VHLD) it tends to recur.[3] Solitary hemangioblastoma for the most part is considered benign and curable by total resection, except in cases associated with VHL disease.[4]

A 42-year-old gentleman presented with complaints of position vertigo since 6 months. There was no history of headache, seizures, vomiting, or weakness in any limb. CNS examination showed positive right cerebellar signs. The Magnetic Resonance Imaging (MRI) Plain and contrast revealed heterogenous signal intensity lesion with few cystic areas and flow voids in the right cerebellar hemisphere with significant enhancement of the solid component. The tumor was measuring approximately 3.5*3.2*3 anteroposterior-craniocaudal-transverse (AP*TR*CC) cm3 causing significant mass effect over the fourth ventricle and brain stem with moderate hydrocephalous and significant perilesional edema. Right retromastoid craniotomy and near total excision of tumor done. The cyst capsule had been removed with the rest of the tumor and a vessel abutting the tumor capsule was purportedly left assuming it to be one of perforators. Histopathology revealed hemangioblastoma (WHO grade 1). Patient had one episode of seizure in ward. His repeat Computed Tomography (CT) scan revealed no hydrocephalous and no swelling over the brainstem.

After 1 month of the first excision procedure, patient reported to the hospital with complaints of three to four episodes of vomiting, diplopia, and giddiness. Redo-exploration and excision procedure of residual hemangioblastoma was performed. Operative findings included bulge brain, thick blood vessels seen as nidus at right CP angle cistern. Post excision of tumor, brain was pulsatile. Preoperative diplopia resolved. Gait imbalance on discharge observed. Followup MRI after one year was normal [Figure 1].
Figure 1: First followup after first surgery

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After 2.5 years of second excision procedure, the patient presented with complaints of right limb weakness and difficulty in walking. MRI [Figure 2] indicated craniotomy defect in right occipital region. Well defined lobulated altered signal intensity lesion noted involving right cerebellar hemisphere. The lesion was intracranial cystic and extracranial solid extended through the craniotomy defect inferiorly outside the skull vault in suboccipital region. The lesion appeared hyperintense on T2, FLARE, and hypointense on T1. Few small prominent feeding vessels noted along the extra solid component. The lesion was closely abutting right sigmoid and transverse and sinus without any evidence of invasion. Mild mass effect and adjacent edema noted in the right cerebellar hemisphere and right middle cerebellar peduncle. Right redo craniotomy and excision procedure of tumor was done. The Recurrence of hemangioblastoma confirmed by histopathology. His post-operative repeat CT brain showed remnant cystic residual component of the solid excised tumor and therefore radiotherapy recommended.
Figure 2: Intracranial cystic component of the lesion measures 3.5*4.1*3.3 (AP*TR*CC) cm3 and the extracranial component of the lesion was predominately solid and showed strong postcontrast enhancement the solid component measured 2.5*4.1*4.0 (AP*TR*CC) cm3

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After a month, based on CT and DOTANOC PET CT, [Figure 3]a the patient was planned for treatment with Cyberknife Robotic Radiosurgery to a dose of 35Gy/5#to GTV PET1, 235 (brain) and 25 Gy/5# to GTV PET125 GTV MR25 (brain) for five days and 6-D skull software was used for tracing the tumor.
Figure 3: (a) is CT scan before CRS and (b) is CT scan after CRS

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Four months later, CT scan revealed mild interval regression in the size of enhancing soft tissue surrounding the surgical cavity with interval regression of metabolic activity. The soft tissue measured 1.1 cm in maximum thickness (previously measuring 1.3 cm). MRI indicated intense enhancement of solid area on postcontrast study. The combined lesion including the surgical cavity measured approximately 3.9*4.8*4.5* cm3 (previously 3.8*5.1*4.4 cm3).

Fourteen months post-CRS, MRI revealed size of the lesion decreased associated with an increase in perilesional edema and gliotic changes involving the right cerebellum [Figure 3]b.

[Figure 4] and [Figure 5] show two years followup MRI, respectively.
Figure 4: Irregular marginated, nodular and peripherally enhancing lesion along the operative site measuring approximately 1.4*1.8*2.4 (AP*ML*CC) cm3

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Figure 5: Irregular marginated, nodular and peripherally enhancing lesion along the operative site measuring approximately 1.2*1.5*2.3 [AP*(medial-lateral) ML*CC*] cm3

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In conclusion, recurrence is extremely rare after gross total resection of SICH which is observed within three years of first surgery where followup after 1.2 years of first surgery was normal. The dose of CRS was unusual indicated consecutive shrinking of tumor, and after two years. Ipsilateral hearing loss after the first CRS is unusual clinical complication [Table 1].[5],[6],[7],[8]
Table 1: Discussion

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This case represents an unusual clinical manifestation, the recurrence of SICH is discussed with management challenges. The patient is independent in daily activities of life and had the right cerebellar signs with gait ataxia.


  Discussion Top


Cerebellar hemangioblastoma is a rare vascular tumor that comprise of 1-2.5% of primary intracranial tumor. The recurrence of the hemangioblastoma are known, more common with VHLD associated cases. Pathology reports of vascular hemangioblastoma after first surgery revealed the multiple fragments of dark brownish, firm tissue measured 2ccs in aggregate along with numerous thin walled vessels with foci of hemorrhage. The stromal cells showed foamy cytoplasm with mild variation in size and shape. It's mitotic activity was low and necrosis was absent. The vessels showed focal hyalinization, This discussion highlights on the pattern of recurrence of hemangioblastoma in the patient along with its clinical manifestation as discussed in [Table 1].

Acknowledgements

We would like to thank Dr. Shraddha Devarshi for general support. Deepest gratitude towards SCES's Indira College Of Pharmacy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Cervio A, Villalonga JF, Mormandi R, Alcorta SC, Sevlever G, Salvat J. Surgical treatment of cerebellar hemangioblastomas. Surg Neurol Int 2017;8:163.  Back to cited text no. 1
  [Full text]  
2.
Kano H, Shuto T, Iwai Y, Sheehan J, Yamamoto M, McBride HL, et al. Stereotactic radiosurgery for intracranial hemangioblastomas: A retrospective international outcome study. J Neurosurg 2015;122:1469-78.  Back to cited text no. 2
    
3.
Yousef A, Rutkowski MJ, Yalcin CE, Eren OC, Caliskan I, Tihan T. Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: Institutional experience on their similarities and differences. J Neurooncol 2019;143:547-52.  Back to cited text no. 3
    
4.
Akimoto J, Fukuhara H, Suda T, Nagai K, Hashimoto R, Michihiro K. Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease. Surg Neurol Int 2014;5:145.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Fukuda M, Takao T, Hiraishi T, Yoshimura J, Yajima N, Saito A, et al. Clinical factors predicting outcomes after surgical resection for sporadic cerebellar hemangioblastomas. World Neurosurg 2014;82:815-21.  Back to cited text no. 5
    
6.
Aziz M, Alam K, Varshney M, Maheshwari V, Sherwani RK, Gaur K, et al. Cerebellar haemangioblastoma: A rare entity. 2011;2011:bcr0320113943.  Back to cited text no. 6
    
7.
Dwarakanath S, Suri A, Sharma BS, Mehta VS. Intracranial hemangioblastomas: An institutional experience. Neurol India 2006;54:276-8.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Inoue HK, Seto K, Nozaki A, Torikai K, Suzuki Y, Saitoh J, et al. Three-fraction Cyberknife radiotherapy for brain metastases in critical areas: Referring to the risk evaluating radiation necrosis and the surrounding brain volumes circumscribed with a single dose equivalence of 14 Gy (V14). J Radiat Res 2013;54:727-35.  Back to cited text no. 8
    


    Figures

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