Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2015  |  Volume : 58  |  Issue : 1  |  Page : 89--92

Central nervous system teratomas in infants: A report of two cases


Shatavisha DasGupta1, Sanghamitra Mukherjee1, Ram Narayan Das1, Anway Sen1, Uttara Chatterjee1, Chhanda Datta1, Sandip Chatterjee2,  
1 Department of Pathology, IPGMER, Kolkata, West Bengal, India
2 Department of Neurosurgery, Park Clinic, Kolkata, West Bengal, India

Correspondence Address:
Dr. Shatavisha DasGupta
15A/7, East Road, Santoshpur, Kolkata - 700 075, West Bengal
India

Abstract

Intracranial teratomas are uncommon neoplasms with most of them being encountered in the pediatric age group. Teratomas are composed of derivatives of all the three germ cell layers and are classified into mature, immature and teratoma with malignant transformation. Two cases of intracranial teratomas in infants are presented here with the idea of highlighting this relatively uncommon condition in an uncommon age.



How to cite this article:
DasGupta S, Mukherjee S, Das RN, Sen A, Chatterjee U, Datta C, Chatterjee S. Central nervous system teratomas in infants: A report of two cases.Indian J Pathol Microbiol 2015;58:89-92


How to cite this URL:
DasGupta S, Mukherjee S, Das RN, Sen A, Chatterjee U, Datta C, Chatterjee S. Central nervous system teratomas in infants: A report of two cases. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Aug 1 ];58:89-92
Available from: https://www.ijpmonline.org/text.asp?2015/58/1/89/151198


Full Text

 INTRODUCTION



Teratomas are fairly common tumors of childhood commonly encountered in retroperitoneum, sacrum, mediastinum and nasopharynx [1] Rarely they can be gastric, intrarenal or intracranial. [2],[3] We came across two cases of intracranial teratomas in infant girls that we present here. This is a tumor rarely reported in infants in Europe or North America, although it has been reported from South East Asia.

 Case Reports



Case 1

A 4-month-old girl was born at full term and delivered by caesarian section. Antenatal ultrasound (US) scans of the mother had failed to pick up any intracranial pathology, although only one scan was performed at 16 weeks. Mother had no folate supplementation during her pregnancy. She was noted to have increasing head circumference at 3 months. She had generalized tonic-clonic convulsions at that age followed by 8 such episodes.

At presentation, she was lethargic and had a bulging anterior fontanelle. US revealed a large, right hemispheric tumor with hydrocephalus. A ventriculoperitoneal shunt was inserted, and she was transferred to us. At admission, she had no eye opening or cry, was flexing to pain on the left side only. Plain computed tomography (CT) scan [Figure 1] confirmed the presence of a left ventricular catheter with a giant right parietal variably-attenuating lesion with cystic changes and calcification. In view of the fact that the child was having seizures, a decision was taken against performing contrast CT scans, and radiological diagnosis was made of an aggressive glioma. Tumor markers in the blood were witih normal limits.{Figure 1}

Right fronto-temporal craniotomy and debulking was done. The child was ventilated for 48 h and then gradually weaned off. On the 10 th postoperative day, she went into status epilepticus. After a stormy period, she expired of intercurrent infections on the 20 th postoperative day.

Microscopic examination of the tumor showed ectoderm, in the form of skin with dermal appendages, mature neuroglia and choroid plexus, mesenchyme in the form of cartilage, bone, muscles (skeletal and smooth) and endoderm in the form of glandular tissue, salivary and intestinal types were noted. On extensive sampling, immature tissue was lacking. A diagnosis of mature teratoma was made [[Figure 2]a-d].{Figure 2}

Case 2

A 3-month-old girl with a history of birth asphyxia presented with failure to thrive. Antenatal US at 24 weeks demonstrated hydrocephalus, but did not comment on any intracranial tumor. She did not have any further followup US examination, although she had been on folic acid supplements all through her pregnancy. Plain and contrast CT scans of the brain revealed a large space-occupying lesion (SOL) with a mixed density occupying most of the right hemisphere [Figure 3], which was diagnosed radiologically to be a teratoma. While in the hospital the baby began to have extension posturing and respiratory difficulty and was intubated. Tumor markers, that is, serum alpha feto-protein (AFP) and human chorionic gonadotrophin (hCG) were sent off, and subsequently found to be negative. Attempted ventricular tap failed and after explaining the prognosis to the parents, a do-or-die craniotomy and debulking was done. The level of consciousness improved during the postoperative period. She was able to flex to pain and had eye opening to pain, but respiratory effort did not return satisfactorily. It was not possible to wean her off ventilatory support. She succumbed due to renal shutdown on the 12 th postoperative day.{Figure 3}

Microscopic examination again showed presence of derivatives of all three germ cell layers. In addition, there were immature neuroblastic foci composed of small round cells with darkly staining nuclei separated by cobweb like neuropil. A few rosettes were also identified. A diagnosis of immature teratoma was made [[Figure 4]a and b].{Figure 4}

 DISCUSSION



Intracranial germ cell tumors are primarily neoplasms of the young. Approximately, 90% afflict those younger than 20 years [4] and the incidence peaks around 10-12 years. [5] These tumors account for 0.4-3.4% of primary pediatric central nervous system (CNS) tumors in the Western population whereas in Japan and Far East the incidence is 7-11%. [6],[7],[8] Intracranial germ cell tumors resemble their gonadal counterparts morphologically, immunohistochemically and cytogenetically.

World Health Organization classifies germ cell tumors into germinomas and non-germinomatous tumors including teratomas (mature, immature and with malignant transformation), yolk sac tumors, embryonal carcinoma, choriocarcinoma and mixed germ cell tumors. [5] Germinoma is the commonest CNS germ cell tumor, forming 65% of intra cranial germ cell tumors (ICGCT). The nongerminomatous group and mixed germ cell tumors form around 20% and 15% respectively. [5] Teratoma constitutes majority of congenital CNS germ cell tumors. [5] Intracranial teratoma forms 0.5% of all intracranial neoplasms yet accounts for the largest proportion of fetal intracranial neoplasms. [9] Accurate histological classification of CNS germ cell tumors is critical for treatment and prognostication.

When CNS as a whole is considered, male: Female ratio for germ cell tumors is 2-2.5:1. Most pineal tumors affect boys, and suprasellar lesions are common in girls. [5] Teratomas have a higher incidence among girls and our cases were girls.

Intracranial teratomas, usually, arise from the suprasellar region, pineal gland, quadrigeminal plate, wall of the third ventricle and cerebellar vermis. [10]

There are two theories regarding their origin. Germ cells first appear during the 4 th week of intrauterine life in the yolk sac and migrate around 6 th week along the dorsal root mesentery to the gonadal ridges. Teilium's theory proposes that extragonadal germ cell tumors arise from the primordial germ cells that migrate in an aberrant fashion and undergo malignant transformation. [10],[11] The alternative embryonal theory states that mismigrated pluripotential embryonic cells give rise to germ cell tumors. [12]

Cytogenetically, an increased predisposition to teratomas is associated with supernumerary X chromosomes. Numerical and structural alterations of chromosome 12 are rare in teratomas, unlike other germ cell tumors. [11]

Clinical manifestations of ICGCT depends on the size and location. [4] Greenhouse and Neubuerger [13] classified the cases clinicopathologically into Group I, comprising still borns with brain totally replaced by tumors; Group II of patients born alive, with hydrocephalus, tumor of intermediate size and Group III formed in babies born healthy, subsequently developing hydrocephalus, having small tumors.

The neuroradiological profiles of ICGCT are largely nonspecific and depend upon the tumor contents. Most of the perinatal tumors are diagnosed antenatally. A rapid growth in maternal girth due to hydramnios is common. Cepahalopelvic disproportion is reported early in the 2 nd trimester. Serial prenatal US demonstrate hydrocephalus prior to the diagnosis of teratoma. A diagnosis of teratoma should be considered for a lesion containing intratumoural cysts admixed with calcified regions and low signal-attenuation, [5] CT and magnetic resonance imaging studies are useful in demonstrating hydrocephalus, invasion of regional structures and cerebrospinal fluid (CSF)-borne metastases. [5]

Assay of serum and CSF oncoproteins is a part of the preoperative evaluation and monitoring response to treatment. The useful markers are AFP, hCG and placental alkaline phosphatase (PLAP). Ratio of CSF: Serum levels of these markers in favor of CSF supports the diagnosis of primary ICGCT.

Macroscopically, teratomas often show mucus-laden cysts, fat, chondroid nodules, or bony spicules, teeth or well-formed hairs. Mature teratomas composed exclusively of fully differentiated, "adult-type" tissues are sometimes arranged in a pattern resembling normal tissue relationship. [5] The level of organization maybe so advanced that a diagnosis of a parasitic twin has to be considered. Immature teratoma is composed of incompletely differentiated hypercellular and mitoticallly active components resembling fetal tissues. [5] Tissues reminiscent of embryonic mesenchyme and primitive neuroectodermal elements are often present. [5] The interpretation of immaturity is mainly restricted to neuroepithelium rather than other elements. Immaturity in the fetal and newborn period is difficult to assess as it may be appropriate for the stage of fetal growth. Scully's grading system, which is used for quantification of immaturity in other locations is not used in the CNS. Microscopically, metastatic Wilms' tumor with its blastema and epithelial components can look similar to immature teratoma. However, it occurs in a different setting. Immature intracranial teratomas have been reported to undergo spontaneous differentiation into mature tissues over time. [14]

Teratoma with malignant transformation is extremely rare and contains a cancer of conventional somatic type, e.g., rhabdomyosarcoma and undifferentiated sarcoma. [4],[15] Immunohistochemistry is important in cranial teratoma mainly to rule out the presence of other germ cell components. Conventional histological techniques, immunohistochemical markers like hCG, AFP, PLAP, cytokeratin, c-kit and OCT4 help in their delineation. [16] The common germ cell markers like PLAP, hCG, OCT4 and SALL4 are negative in most teratomas. However, cytokeratin immunoreactivity is a feature of the epithelial component, and AFP expression is, usually, restricted to glandular components. [5] The single most predictive feature is the histological subtype. [6] Pure germinoma has a favorable prognosis owing to its remarkable radiosensitivity. [5] Patients harboring nongerminomatous germ cell tumors are associated with poor outcome with the exception of those who can tolerate gross total resection like fully mature teratomas, which tend to be noninvasive. [5] Recent results suggest that the chemotherapy in conjunction with craniospinal radiotherapy can improve survival. Majority of reports contain information about only a few patients, which highlights the relative rarity of these tumors and the difficulty in designing the best treatment. There is no doubt that few infants with these tumors survive to their first birthday.

 CONCLUSION



The diagnosis of intracranial teratoma should be borne in mind for infants presenting with giant intracranial SOLs. Extensive sampling is required to rule out the presence of additional malignant components.

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