|Year : 2015 | Volume
| Issue : 2 | Page : 126-128
Congenital renal teratoma with cystic dysplasia in a neonate: Rare entity
Jayesh Modi1, Deepika Modi2, Mitesh Bachani3, Lira Bachani2
1 Department of Urology and Renal Transplantation, Smt. Gulabben Rasiklal Doshi and Smt. Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre and Dr. H. L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India
2 Department of Pathology, Unipath Diagnostic Centre, Ahmedabad, Gujarat, India
3 Department of Pediatric Surgery, B.J. Medical College, Civil Hospital, Ahmedabad, Gujarat, India
|Date of Web Publication||6-Apr-2015|
Dr. Jayesh Modi
2, Kamdhenu Apt., Behind St. Xaviers Loyolla Hall School, Memnagar, Ahmedabad - 380 052, Gujarat
Source of Support: None, Conflict of Interest: None
Germ cell tumors rarely originate from the kidney. We here present a case of primary intrarenal mature teratoma with renal dysplasia associated with mid penile hypospadias in 26-day-old child. Our case is unusual due to the younger age of child and association of renal dysplasia with primary renal teratoma.
Keywords: Dysplasia, extragonadal, kidney, mature, teratoma
|How to cite this article:|
Modi J, Modi D, Bachani M, Bachani L. Congenital renal teratoma with cystic dysplasia in a neonate: Rare entity. J Clin Neonatol 2015;4:126-8
|How to cite this URL:|
Modi J, Modi D, Bachani M, Bachani L. Congenital renal teratoma with cystic dysplasia in a neonate: Rare entity. J Clin Neonatol [serial online] 2015 [cited 2019 Sep 17];4:126-8. Available from: http://www.jcnonweb.com/text.asp?2015/4/2/126/154123
| Introduction|| |
Germ cell tumors rarely originate from the kidney. Teratoma by definition includes structures derived from all three embryonic layers: Ectoderm, endoderm, and mesoderm. Most common sites of teratoma are sacrococcygeal, ovary, testicle, pineal gland, and retroperitoneum. Primary renal teratoma is extremely rare. We here describe a case of primary renal mature teratoma associated with renal dysplasia and hypospadias in a 28-day-old male child which is even more unusual.
| Case Report|| |
A 26-day-old male child was brought by mother with complain of swelling in the right side of abdomen and increase crying associated with passing urine through ventral surface of penis. She also gave a history of fever for 2 days. The birth weight of the child was 2.1 kg with history of full term vaginal delivery. The antenatal ultrasound was done at 30 weeks by local doctor, and the mother was told that some problem in the kidney of baby and was advised to consult pediatric surgeon after birth. On examination, the child was irritable with dehydrated and palpable firm right renal lump. The genitalia were normal except mid penile hypospadias. His blood pressure was normal. On investigations, his hemoglobin, creatinine and liver function tests were normal. Computed tomography revealed enlarged right kidney with lobulated, heterogeneous, multiple hypodense areas [Figure 1]a & b So in view of infective or neoplastic lesion of the right kidney, the decision was taken for nephrectomy. Right open retroperitoneal nephrectomy was done. On gross examination, the kidney was 7 cm × 6 cm × 3 cm in size with 5 cm of ureter. The cut surface shows multilocular cystic and solid areas with a thin rim of the renal parenchyma at lower pole [Figure 2]. The microscopy revealed ectodermal, endodermal and mesodermal elements. The teratomatous components consist of keratinizing stratified squamous epithelium with skin adnexae, glands, cartilage and trabeculae of bone [Figure 3]a. Dysplastic elements were primitive glomeruli and tubules with surrounding cellular stroma [Figure 3]b. The final diagnosis was made as primary renal mature teratoma with dysplasia. On 1 year follow-up child is alive without any recurrence with normal tumor markers.
|Figure 1: (a) CT film shows right side enlarged kidney with multiple hypo dense and solid areas with thin renal parenchyma. Also, shows normal left kidney, (b) CT film shows arterial phase. Contrast is seen in aorta. Right side enlarged kidney with multiple hypo dense and solid areas|
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|Figure 2: Gross view of the right kidney shows multilocular cystic and solid areas with the thin rim of the renal parenchyma present at the lower pole|
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|Figure 3: (a) Islands of immature cartilage and squamous epithelium with glands set in a fi brous stroma (H and E, ×10), (b) Primitive glomeruli and immature appearing tubules with surrounding cellular stroma (H and E, ×10)|
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| Discussion|| |
Teratomas are germ cell tumors and commonly arise in the gonads and less frequently from the median line of the body such as in the mediastinum, retroperitoneum, sacrococcygeal region, and pineal gland.  Sacrococcygeal region is more commonly involved in infants. Teratomas and other germ cell tumors rarely originate primarily from kidney. 
In 1934, McCurdy described the first case of teratoma of kidney in a 7-week-old child with prune belly syndrome.  Since then, about 30 cases of primary intrarenal teratomas have been found in English literature.  Primary renal teratoma associated with dysplasia is even rare occurrence, with very few reported cases. ,
Choi et al.  reviewed 20 cases of primary renal teratomas from 1934 to 2005. Out of them, 12 cases were found in children (60%) and 8 (40%) in adults (average age, 17 years; median age, 3 years). The male-to-female ratio was about 1:1.4. Histologically, 15 teratomas (75%) were of immature histologic grade, and of these tumors, just <50% were locally infiltrating or metastatic. Five cases (25%) were associated with congenital abnormalities (horseshoe kidney, duplicated collecting system, prune belly syndrome and oligodactyly-like syndrome) suggesting that the mal development increases the risk of teratoma. In our case, hypospadias was associated anomaly.
Several hypotheses of teratoma origin have been suggested, namely: (i) The germ-cell theory; (ii) the embryonic-cell theory; (iii) the unifying hypothesis; (iv) the extra-embryonic-cell theory; (v) the induced-twin hypothesis; and (vi) the fetus-in-fetus theory.  The proximity of the genital ridge to the nephrogenic anlage during embryogenesis may partly explain presence of displaced germ cells into the kidney.  Few cases of renal teratoma were found to be associated with renal dysplasia, which may suggest that intrarenal teratoma is associated with development. 
The clinical symptoms are an abdominal mass, abdominal pain, anorexia, vomiting, constipation, and hematuria.  Plain X-ray may show calcification or bony components like teeth. Serum alpha fetoprotein is an important marker for followup to detect recurrence.  Hayasaka et al. have described that internal homogeneity, fat density; cyst formation and calcification are important predictors of benign retroperitoneal tumor on computed tomography scan.  The role of magnetic resonance imaging in renal teratoma is not clear. Papanicolaou et al. have described angiography, inferior venacavography and needle biopsy for the accurate diagnosis of retroperitoneal teratomas.  Serum alpha-fetoprotein formed a useful marker of monitoring recurrence.
Differential diagnosis includes multicystic renal dysplasia, hydronephrosis, infected renal cysts, Wilms' tumor and neuroblastoma. The renal teratoma must be differentiated from teratoid variant of Wilms' tumor. The latter consists predominantly of heterogeneous tissues such as bone, cartilage, muscle, fat, neuroglial tissue, and mature squamous epithelium. For a tumor to be termed a primary renal teratoma, Beckwith suggested two criteria: (i) The primary tumor should be unequivocally of renal origin, means there should be no teratomas in remote sites which may have metastasized to the kidney and (ii) the tumor should exhibit unequivocal heterotopic organogenesis, with clearly recognizable evidence of attempt to form organs other than kidney.  Rarely, this tumor presented antenatally and diagnosed later.
Teratomas are divided into mature and immature types. Mature teratoma mainly contains well-differentiated tissues like dermal appendages, skin, neurological tissue and teeth, while immature teratoma contains mature epithelium and immature areas of neuroectodermal and mesenchymal tissue. Mature teratomas are, usually, benign, and metastasis from intrarenal teratoma has not been reported till now. However, metastasis form mature teratoma of various other origins has been described.  Risk of malignancy is higher in children presenting in early age as compared to older children.  So, surgical excision is justified. Features suggest poor prognosis are immature histologic grade; association with malignant neuroepithelial tumors and presence of metastases. 
The aim of highlighting this case is that primary renal teratoma should be kept in the differential diagnosis of infantile renal masses, though rare. Our case is unusual due to the younger age of child and association of renal dysplasia and hypospadias with primary renal teratoma.
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[Figure 1], [Figure 2], [Figure 3]