Case Reports: Management Challenges in Pediatric Germ Cell Tumors

Abstract

Germ cell tumors in infants are most frequently extragonadal, benign, and amenable to surgical resection. An unusual feature of germ cell tumors is the potential coexistence of malignant with benign disease which makes it possible for patients with incompletely resected tumors to experience either a benign or malignant recurrence. A challenge to postoperative surveillance is the interpretation of serum alpha fetoprotein, a marker of malignancy, that is physiologically elevated during the first year of life. A rare subset of germ cell tumors occur in the retroperitoneum. Although the vast majority are benign, these tumors are often large and distort normal anatomy, and may demonstrate local invasiveness that increases risk of resection. The intent of these reports is to caution readers about these unusual features of germ cell tumors of infancy.

Giant malignant sacrococcygeal germ cell tumor in a newborn: A rare case report

“…Although SCTs are classified according to the absence (grade 0) and the amount of immature tissue (grades 1, 2, 3) the histological grade of immaturity is not correlated with prognosis and only the presence of yolk sac tumor elements is predictive of malignant behavior. We found a grade 3 immature teratoma with yolk sac components, confirming, however, the known association between high-grade neoplasms and microscopic foci of malignancy [1] , [2] , 6 , 10] . Elevated serum AFP is considered the best indicator of yolk sac tumor, but this association must be interpreted carefully as the AFP is physiologically high in newborns because of fetal production and gradually declines to the normal adult range by 8 months after birth.…”

“…Our little patient was considered to have elevated AFP levels for age consistent with the diagnosis and decreased quickly following surgery. After resection of SCT, AFP has a good predictive value for the presence of residual disease and its serial measurement is useful in the follow-up as, in absence of recurrence, it should fall and normalize within 6 to 12 months (9 months on average) [2 , [4] , [5] , [6] , [7] , [10] , [11] . Clinical presentation varies depending on the size and location of the SCTs.…”

“…As in our case, the MRI is primarily used for evaluation of intraspinal invasion and for excluding the meningocele and myelomeningocele which are birth defects, grouped together under the term spina bifida cystica, looking like a sac filled with fluid leading out from the newborn's spine. Moreover, MRI is the modality of choice for the detection of neoplastic recurrence during follow-up, as it does not expose children to ionizing radiation and provides superior soft-tissue characterization to CT, exploiting the properties of magnetic fields to obtain images with high contrast resolution, acquired directly in 3 dimensions [6] , [7] , [9] , [10] , [13] , [14] , [15] , [18] , [19] . SCTs appear as complex masses, containing variable amounts of solid heterogeneous and cystic areas with or without septations, and imaging findings alone cannot differentiate between benign and malignant lesions.…”

“…We adopted the “wait and watch” policy and chemotherapy will be initiated if there is evidence of reappearance of the disease based on rise in AFP and/or the presence of neoplastic tissue at MRI [1 , 20] . The recurrence rate of malignant teratoma is 0%-36% (mean, 18%) and the most important risk factors are incomplete resection of the neoplasm and/or coccyx, and/or tumor rupture/spillage during the cesarean delivery or surgical removal [4] , [5] , [6] , [7] , [8] , 10 , 12] . The probability of relapse of the disease is high in the first 3 years after surgery, but our little patient will undergo close follow-up for at least 5 years with frequent AFP level measurements alternated with diagnostic imaging examinations (abdominal ultrasound, chest x-ray, and pelvic/spinal MRI) [4 , 20] .…”

Neonate with Retroperitoneal Mature Cystic Teratoma – A Case Report

“…3 The patients with retroperitoneal teratomas have a female preponderance, but conversely, our patient was a male neonate with a teratoma which was located at the retroperitoneum. 4 They are usually secondary neoplasms and primary retroperitoneal neoplasms are extremely rare. 1 The anatomic variety seen in these tumours and their occurrence in midline and gonads is explained by the germ cells migratory capacity.…”