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Table of Contents    
LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 6  |  Page : 1855-1858

A Rare Case Presentation of Supratentorial PNET in a Pregnant Lady


1 Department of Neurosurgery, Maharaja Agrasen Medical College, Agroha, Haryana, India
2 Department of Radiology, Maharaja Agrasen Medical College, Agroha, Haryana, India
3 Department of Pathology, Maharaja Agrasen Medical College, Agroha, Haryana, India
4 Department of Anesthesia, Maharaja Agrasen Medical College, Agroha, Haryana, India
5 Department of Genral Surgery, Maharaja Agrasen Medical College, Agroha, Haryana, India

Date of Submission30-Jun-2019
Date of Decision04-Nov-2019
Date of Acceptance17-Oct-2020
Date of Web Publication23-Dec-2021

Correspondence Address:
Dr. Ishu Bishnoi
Assistant Professor, Neurosurgery, Maharaja Agrasen Medical College, Agroha - 125 047, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.333496

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How to cite this article:
Bishnoi I, Singh P, Singh K, Duggal G, Kumar S, Bhardwaj L. A Rare Case Presentation of Supratentorial PNET in a Pregnant Lady. Neurol India 2021;69:1855-8

How to cite this URL:
Bishnoi I, Singh P, Singh K, Duggal G, Kumar S, Bhardwaj L. A Rare Case Presentation of Supratentorial PNET in a Pregnant Lady. Neurol India [serial online] 2021 [cited 2022 Jan 19];69:1855-8. Available from: https://www.neurologyindia.com/text.asp?2021/69/6/1855/333496




Dear Sir,

Primitive neuroectodermal tumors (PNETs) are tumors of round, poorly differentiated cells with high malignant potential.[1] In adults (more than 20 years age), they constitute 0.46% of all cranial tumors.[1] Less than 100 cases of adult supratentorial PNETs have been reported so far and out of all these cases, only two case reports are about pregnancy with PNET tumor.[2],[3] We are reporting third case of Supratentorial PNET in pregnant lady, management of both tumor and pregnancy.

24 years primiparous, Gravida1 was brought with complaints of left side headache, vomiting for four months and right hemiparesis for one month. She had 4 months pregnancy and her fetal ultrasound was normal. On examination, her right upper limb and lower limb power was 4/5. MRI brain revealed left frontal solid-cystic mass with perilesional edema [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f. The differential diagnoses were high-grade glioma, metastasis and infective etiology.
Figure 1: (a) MRI brain T1 axial cut showing multicystic lesion left frontal lobe, (b) T2 sagittal cut showing solid portion with multiple cyst around it, reaching till corpus callosum and causing edema of surrounding, (c) T2 coronal cut showing compression of ipsilateral ventricle, well defined lesion, (d) contrast enhanced T1 axial cut, sagittal cut (e), coronal cut (f) showing enhancement of tumor margins and solid portion, (g) intra-operative image showing well defined lesion (blue star), surrounding edema (yellow dot), (h) showing cysts (yellow arrow) with well-defined plane of dissection

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The patient underwent surgical decompression on semi-emergency basis due to doubt of high-grade lesion.

After informed consent, she underwent left frontal craniotomy. The obstetrician was kept in back up. The tumor was multicystic [Figure 1]g, pinkish gray, vascular, having well defines plane of dissection [Figure 1]h and significant brain edema. Gross total resection was achieved.

She recovered well and monitored in ICU for two days. MRI brain revealed complete removal of tumor [Figure 2]c,[Figure 2]d,[Figure 2]e,[Figure 2]f. The fetal heart sound was normal. She was discharged uneventfully. CSF study, after 20 days, was negative for malignant cells.
Figure 2: Postoperative MRI T1 (a) axial cut showing complete resection, air (dark colored on T1) fluid (hypointense on T1) level. (b) axial cut showing complete resection, air (dark colored on T2) fluid (hyperintense on T2) level, (c) FLAIR axial cut showing reduced edema of motor strip area, (d) T1 and (e) T2 coronal cut showing complete resection of lesion, (f) T2 sagittal cut showing complete resection, air-fluid level, shrunken brain, (g) histopathology slide of tumor cells, showing distinct membrane positivity with CD99, (h), (i) histopathology slides (400×) showing round tumor, scant cytoplasm, with mitotic activity and anaplastic features

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The tumor cells had round cells, scant cytoplasm, with mitosis and anaplasia [Figure 2]g and [Figure 2]h. The cells had membrane positivity with CD99 [Figure 2]i, negative for PDGFR-alpha, PDGFR-beta and c-kit.

She delivered full term, healthy baby girl by normal vaginal delivery. Her birth weight was 2700 g and APGAR score 9/10. The mother and fetus were discharged on second day.

Patient was regularly followed up. She refused chemotherapy and radiotherapy. Contrast enhanced MRI brain with MRS (magnetic resonance spectroscopy) after 1.5 years revealed no recurrence, a contrast enhancing dural nodule (7 mm size) and gliosis [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d,[Figure 3]e,[Figure 3]f. MRS of dural nodule was inconclusive. MRI spine and CSF study were normal. The levetiracetam was stopped after 1 year.
Figure 3: (a) MRI brain FLAIR, axial cut showing postoperative changes in left frontal lobe near midline, (b) T2 coronal cut showing gliosis, left frontal lobe, paramedian location, with upward traction of left lateral ventricle, (c) T2 sagittal cut showing well circumscribed dural nodule, 7 mm size at surgery site, (d) contrast T1 axial cut showing enhancement of nodule, (e) contrast T1 axial deeper cut showing no enhancement of parenchyma at surgery site, (f) contrast T1 sagittal showing in contrast enhancement of dural nodule, projecting in scarred brain at tumor site

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Hajoun et al. have reported features of PNET on MRI, which are large size; solid-cystic components, well-defined margins, occasional intra tumoral hemorrhage and calcification.[4] The MRI features of our case were matching with Hajoun et al. study.

Bodner-Adler B et al. first reported about PNET in pregnancy.[2] They did near total resection. Radiation and chemotherapy were given during pregnancy. The fetus developed growth retardation and delivered early by cesarean. Ulivieri et al. reported second case of pregnancy with PNET.[3] They also left residue. The fetus was delivered early by cesarean and second surgery was done after it, followed by chemo-radiation. Because of favorable factors i.e. frontal lobe resection, superficial location, we achieved gross total resection. As patient refused chemo-radiation, pregnancy was allowed its natural course.

In PNETs, postoperative lumbar puncture CSF study is an important investigation to detect metastasis along craniospinal axis Negative sample rules out metastatic disease, while positive sample is suggestive of metastasis.

The Supratentorial PNET is a rare tumor. Due to its unique presentation on contrast MRI, it may be considered as a differential. There are few factors, which caused good prognosis in our patient – frontal lobe location, well-defined margins and aggressive management i.e. complete surgical resection. We recommend normal delivery of baby, if not contraindicated otherwise.

Acknowledgement

We are grateful to staff Riya, Kavita, Varsha Patyal for assisting the case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Amita R, Sandhyamani S, Nair S, Kapilamoorthy TR. Intracranial ewings sarcoma/peripheral primitive neuroectodermal tumor. Neurol India 2014;62:432-3.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Bodner-Adler B, Bodner K, Zeisler H. Primitive neuroectodermal tumor (PNET) of the brain diagnosed during pregnancy. Anticancer Res 2006;26:2499-501.  Back to cited text no. 2
    
3.
Ulivieri S, Oliveri G, Filosomi F, Petraglia F. Supratentorial primitive neuroectodermal tumor during pregnancy. Case report. Minerva Ginecol 2007;59:199-201.  Back to cited text no. 3
    
4.
Hajoun S, Xiangquan K, Haibo X, Liying X, Dingxi L. MRI features of intracranial primitive neuroectodermal tumours in adults: Comparing with histopathological findings. J Huazhong Univ Sci Technol 2004;24:99-102.  Back to cited text no. 4
    


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