Fetus in Fetu: a Fetiform Teratoma

Examination of a retroperitoneal fetus in fetu, diagnosed preoperatively, revealed previously unreported histologic findings in the vascular pedicle and membranous capsule that indicated that these structures are not “umbilical cord” or “amnion.” These findings include nervous innervation of both structures and a well-defined lamina elastica interna and vaso vasorum in the artery of the vascular pedicle. Thus, strong support is provided for the theory that many examples of fetus infetu are remarkably complex, well differentiated, highly organized teratomas. Additional arguments that favor the identity of fetus in fetu and teratoma are presented.     

The Origin of Extragonadal Teratoma: Case Report of an Immature Teratoma Occurring in a Prenatal Brain

We describe a massive congenital intracranial teratoma (MCIT), which had a normal chromosome banding pattern 46,XY karyotype and a normal diploid DNA histogram, and which produced a variety of carcinoembryonic antigens. The volume density of primitive neural components (primitive neural tubes, small undifferentiated neuroepithelial cells, immature glial fibers and pigment cell components without neurofibrillar differentiation) was estimated to be 45%. We discuss the histogenesis, pathobiology and cell cycle kinetics.     

Intracardiac teratoma in an infant

Summary We report the only infant known to us who has survived surgery for a primary right ventricular teratoma. Included is a review of other reported cases of teratoma of the heart in infancy and childhood.     

Endodermal Sinus Tumor of the Parotid Gland in a Child

We report a case of a 16-month-old girl with a primary endodermal sinus tumor (EST) of the parotid gland. The girl was admitted to the hospital with a left side cervical tumor with a quick growth (within 3 wk). The tumor was surgically resected. The gross examination showed a 7.0-cm hemorrhagic and fragmentized mass. Diagnosis of an EST was established on the hematoxylin and eosin-stained slides from the resected material. Elevated serum alpha-fetoprotein (AFP) levels were detected and follow-up examinations of the patient did not show ESTs in other locations or other manifestations due to the tumor. The patient received chemotherapy after the surgery and remained alive without evidence of disease at the time of this writing (2 years after the diagnosis). As far as we know, this is the second report in the literature of a parotid glands EST.     

Successful Treatment of a Mediastinal Endodermal Sinus Tumor in a 2-Year-Old Child

The article describes a primary mediastinal endodennal sinus tumor in a child. The employment ofmultidrug therapy combined with surgical treatment resulted in a long-term remission in this rare tumor.     

Metastatic Intracranial Chordoma in a Child with Massive Pulmonary Tumor Emboli

A 27-month-old boy of Hispanic background developed multiple cranial nerve palsies, difficulty swallowing, bloody nasal discharge, and irritability. Radiographic evaluations showed extensive destruction of the clivus by a large tumor that invaded the sphenoid bone, left cavernous sinus, ethmoid sinus, nasal cavity, and left orbit. Multiple pulmonary nodules were also noted. The bone marrow and spinal fluid showed no evident tumor cells. Transnasal biopsy revealed a chordoma. Treatment was initiated with a combination of ifosfamide, mesna, and etoposide along with radiation therapy to the cranial tumor. Shifting pulmonary densities were noted on serial films. Despite some clinical improvement, the child developed rapidly progressive hypoxemia 3 weeks after admission and died. Autopsy showed persistent viable tumor in the primary site and massive pulmonary arteriolar tumor emboli, infarcts, and widespread lung parenchymal metastases. No other sites of tumor involvement were discovered. This is the second child reported with intracranial chordoma, pulmonary metastases at diagnosis, and early death attributed to pulmonary tumor emboli.     

Improvement of Left Ventricular Function After Changing the Pacing Site in a Child with Isolated Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy

We report a case of isolated congenital complete atrioventricular block with left ventricular dysfunction after pacemaker implantation that improved after the pacing site was changed. During the neonatal period, a pacemaker wire was implanted on the right ventricular epicardium and pacing was initiated. Decreased ejection fraction and a perfusion defect around the septum on myocardial scintigraphy were observed during follow-up. Induced left bundle branch block was thought to be causing interventricular asynchrony, and the pacing site was change to the left ventricular epicardium. Ejection fraction improved and the perfusion defect resolved. Lead relocation may be useful for left ventricular dysfunction that develops during right ventricular pacing.     

Pathologic Characterization of a Renin-Secreting Juxtaglomerular Cell Tumor in a Child and Review of the Pediatric Literature

We describe a rare case of renal hypertension in a 15-year-old caused by juxtaglomerular cell tumor and compare our findings with those of 20 children reported in the literature. These tumors are usually encapsulated and composed microscopically of polyhedral cells with bland nuclei separated by fibrovascular septa. Characteristic renin granules can be demonstrated by Bowie's stain or electron microscopy. These tumors are benign. Pathologists should recognize the morphologic characteristics of these tumors when dealing with renin-producing neoplasia.     

Castleman's Disease of the Left Triceps in a Child Suspected to Be a Small Round Cell Tumor of Childhood

Castleman's disease (CD) is histologically characterized by a proliferation of polyclonal small lymphocytes and plasma cells. The clinical presentation varies widely, but most commonly manifests as a solitary mediastinal mass, incidentally found on radiographic examination. We present a case of a 10-year-old girl who exhibited a left arm mass which, preoperatively and on frozen section, was diagnosed as a small round cell tumor of childhood (SRCT). This report emphasizes the unusual location of CD in the soft tissue and as a rare entity to be considered in the differential diagnosis of SRCT. Received January 15, 1999; accepted July 12, 1999.     

Dysgerminoma in Mother and Daughter: Use of Lactate Dehydrogenase as a Tumor Marker in the Child

A 7-year-old girl presented with an extragonadal dysgerminoma arising from the pelvis. Her mother had been treated for a histologically identical pituitary tumor 3 years previously. The child's serum lactate dehydrogenase (LDH) level was markedly elevated at presentation and fell as the tumor responded to treatment. The potential use of LDH as a marker for gonadal dysgerminoma is well documented, but raised LDH in association with primary extragonadal dysgerminoma has not been described previously. In addition, this is the first report of extragonadal dysgerminoma occurring in female relatives.     

Dysgerminoma in Mother and Daughter: Use of Lactate Dehydrogenase as a Tumor Marker in the Child

A 7-year-old girl presented with an extragonadal dysgerminoma arising from the pelvis. Her mother had been treated for a histologically identical pituitary tumor 3 years previously. The child's serum lactate dehydrogenase (LDH) level was markedly elevated at presentation and fell as the tumor responded to treatment. The potential use of LDH as a marker for gonadal dysgerminoma is well documented, but raised LDH in association with primary extragonadal dysgerminoma has not been described previously. In addition, this is the first report of extragonadal dysgerminoma occurring in female relatives.     

Primary Ovarian Malignant Melanoma Arising in Teratomatous Component of Mixed- Germ Cell Tumor in a Child: Case report

Primary ovarian malignant melanoma arising in teratomatous component of germ cell tumors is seen extremely rare with most reports being only of single cases and small series in reproductive aged woman and mostly from cystic teratoma, whereas information on pediatric presentation is sparse. This case is reported for being extremely rare tumor.     

Malignant Triton Tumor Presenting as a Rectal Mass in an 11-Month-Old

We report the case of an 11-month-old white male who had a family history of neurofibromatosis, had multiple café-au-lait spots on the trunk and extremities, and was diagnosed with a malignant triton tumor of the rectum. To our knowledge, this is the first report of a malignant triton tumor of the rectum and one of the youngest patients reported with the tumor.     

Giant Cell Tumor in the Skull of a 9-year-old Child: Immunohistochemistry to Confirm a Diagnosis Rare for Age and Site

Giant cell tumor of the bone is usually located within the epiphysis of a long bone, the majority of the lesions occurring in the third and fourth decades of life. We report an unusual case of giant cell tumor (GCT) arising in the parietal skull bone of a 9-year-old girl. The tumor exhibited histologic findings typical for GCT, with conspicuous intravascular giant cells. Based on microscopic features, not only conditions like aneurysmal bone cyst or bone changes associated with hyperparathyroidism but also tumors such as chondroblastoma or osteosarcoma had to be considered. Immunohistochemistry revealed strong reactivity of the tumor giant cells and normal bone osteoclasts with CD68 but not Mac-387; tumor stromal cells were uniformly negative for both. The stromal cells exhibited two immunohistochemically distinct phenotypes. One, involving 50-80% of the tumor cells, exhibited negative lysozyme staining with positivity of proliferating cell nuclear antigen (PCNA) in about 30% of the nuclei. The other showed reactivity with lysozyme but negative PCNA staining. Immunohistochemistry thus helped to distinguish chondroblastoma and osteosarcoma, in which lysozyme positivity would reside in macrophages but not within stromal cells. Instead, chondroblastoma would exhibit protein S-100 positivity in the tumor cells. The biological behavior of GCT is difficult to predict based on morphology alone, although the malignant potential seems to rest in the stromal cells rather than the giant cells. Specifically, in reported cases, the intravascular occurrence of giant cells in GCT is not associated with an increased incidence of metastasis.     

Enrichment of Residual Tumor Cells in Bone Marrow Or Peripheral Blood Cells in a Neuroectodermal Tumor Model

To enrich a small population of malignant cells contaminating human bone marrow or peripheral blood, a method of immunomagnetic depletion of the normal nucleated cells was developed. In a model using neuroectodermal cell lanes, contaminations between 0.1% and 1.4% of tumor cells could be increased by a factor of 7.5 (median; range, 3.8 to 18.0; n = 10). Because incubation with antibodies is restricted to the magnetic beads in this method, th cell population after removal of the beads has not been incubated with antibodies. The risk of unspecific staining of the tumor cells during the enrichment procedure is minimal. This simple method is therefore well suited to the study of the characteristics of a minimal residual disease population.     

Enrichment of Residual Tumor Cells in Bone Marrow Or Peripheral Blood Cells in a Neuroectodermal Tumor Model

To enrich a small population of malignant cells contaminating human bone marrow or peripheral blood, a method of immunomagnetic depletion of the normal nucleated cells was developed. In a model using neuroectodermal cell lanes, contaminations between 0.1% and 1.4% of tumor cells could be increased by a factor of 7.5 (median; range, 3.8 to 18.0; n = 10). Because incubation with antibodies is restricted to the magnetic beads in this method, th cell population after removal of the beads has not been incubated with antibodies. The risk of unspecific staining of the tumor cells during the enrichment procedure is minimal. This simple method is therefore well suited to the study of the characteristics of a minimal residual disease population.     

Is There a Place for Chemotherapy in the Management of Recurrent Digital Fibrous Tumor of Childhood? A Case Report

The case of an 11-year-old boy suffering digital fibrous tumor of childhood with multiple recurrences is presented. The possible benefit of chemotherapy instead of a high-level amputation is discussed.     

Dissection of an Aneurysmic Ascending Aorta in a Child with Atresia of the Aortic Isthmus

A boy with atresia of the aortic isthmus suffered dissection of the aneurysmatic ascending aorta. It is assumed that he had coarctation as a newborn and survived the subsequently developed atresia because of extensive collateralization.