A Rare Case Presentation of Supratentorial PNET in a Pregnant Lady
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.333496
Source of Support: None, Conflict of Interest: None
Primitive neuroectodermal tumors (PNETs) are tumors of round, poorly differentiated cells with high malignant potential. In adults (more than 20 years age), they constitute 0.46% of all cranial tumors. 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., 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.
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.
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.
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. The MRI features of our case were matching with Hajoun et al. study.
Bodner-Adler B et al. first reported about PNET in pregnancy. 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. 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.
We are grateful to staff Riya, Kavita, Varsha Patyal for assisting the case.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3]