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Teratoid Wilm's tumor with cryptorchidism


 Department of Pathology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

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Date of Submission06-Nov-2021
Date of Acceptance22-Nov-2021
Date of Web Publication26-May-2022
 


How to cite this URL:
Garg N, Selhi PK, Soni A. Teratoid Wilm's tumor with cryptorchidism. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Feb 5]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345850




A 5-year-old boy presented with an abdominal mass in the left flank region, which was noticed one month ago. On examination, a lump was palpable in the left lumbar region. Also, the right testis could not be palpated in the right scrotal sac. Ultrasonography abdomen revealed a heterogeneous mass at the lower pole of the left kidney. Fine needle aspiration cytology (FNAC) showed a triphasic tumor composed of sheets of round cells having fine nuclear chromatin and scant cytoplasm, i.e., blastemal component, few tubules of epithelial cells along with fragments of the myxoid stroma. Thus, a cytological diagnosis of this being Wilm's tumor was rendered. A partial nephrectomy was performed after four cycles of neoadjuvant chemotherapy. Ultrasonography scrotum showed the presence of left testis having normal outline and echotexture. However, the right testis could not be visualized in the right scrotal sac and right inguinal canal.

Grossly, the left partial nephrectomy specimen measured 9 × 5 × 5 cm with the presence of a well-circumscribed tumor at the lower pole measuring 7 × 3.3 × 2.5 cm. Areas of hemorrhage and necrosis were evident. However, no invasion into the renal sinus or renal vessels was identified. Histological examination revealed a well-circumscribed tumor tumour [[Figure 1]; panel A] composed mainly of heterologous mesenchymal components seen as islands or cartilage, osteoid, and mature neuroglial tissue [[Figure 1]; panel C and D]. The focal area (<15%) showed the presence of epithelial components seen as tubules lined by cuboidal cells [[Figure 1]; panel B]. No blastemal component or anaplasia was identified. Also identified were areas of hemorrhage, necrosis, and haemosiderin-laden macrophages, i.e., chemotherapy-related changes. Histopathological findings led to the diagnosis of teratoid Wilm's tumor.
Figure 1: Photomicrograph (Haematoxylin and eosin stain; x40) shows a well-circumscribed tumour (panel A); Photomicrograph (Haematoxylin and eosin stain; x100) shows tubules of epithelial cells (black arrow) with interspersed mature neuroglial tissue (panel B); Photomicrograph (Haematoxylin and eosin stain; x100) shows mature neuroglial tissue (blue arrowhead) and cartilage (red asterisk) (panel C); Photomicrograph (Haematoxylin and eosin stain; x100) shows osteoid (panel D)

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Wilm's tumor is one of the commonest renal neoplasms of childhood that arises from the totipotent cells of mesonephric blastema, which has the potential to differentiate along epithelial as well as mesenchymal pathways. Teratoid Wilm's tumor (TWT) is a rare variant of Wilm's tumor with uncertain pathogenesis, wherein heterologous components (epithelial or mesenchymal) comprise >50% of the tumor area. Fernandes et al.[1] described teratoid Wilm's tumor (TWT) as having the presence of a heterologous component occupying >50% of tumor area. The heterologous component may be epithelial or mesenchymal in origin. The epithelial heterologous component may be seen as squamous epithelium, mucinous columnar epithelium, respiratory epithelium, etc., The heterologous mesenchymal component can be seen as the presence of cartilage, bone, neural tissue, smooth muscle, skeletal muscle, and rhabdomyoblasts. Differential diagnoses of TWT include renal teratoma, metastasis from a germ cell tumor, and angiomyolipoma.[2] Teratoma and TWT may have similar histology but teratomas usually display unequivocal organogenesis.

As TWT is a rare entity, the role of neoadjuvant chemotherapy is not well-established in such cases yet. However, Children Oncology Group (COG) has proposed guidelines for both preoperative and postoperative chemotherapy.[3] The presence of heterologous components leads to chemotherapy resistance due to the presence of mature teratoid components. Yet, a complete cure can be achieved only when the tumor is completely excised by surgery. Tumor histology is one of the important prognostic factors. Of all, the presence of anaplasia qualifies for unfavorable histology leading to resistance to chemotherapy.[3]

In conclusion, we want to highlight that clinicians and pathologists should be aware of teratoid Wilm's tumor (TWT) for optimal treatment. The treatment of choice for TWT should be surgical excision and chemotherapy should be avoided in the cases with stage 1 disease with favorable histology (no anaplasia) to avoid chemotherapy-related complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Fernandes ET, Parham DM, Ribeiro RC, Douglass EC, Kumar AP, Wilimas J. Teratoid wilms tumor: The St Jude experience. J Pediatr Surg 1988;23:1131-4.  Back to cited text no. 1
    
2.
Ghamdi DA, Bakshi N, Akhtar M. Teratoid Wilms tumor: Report of three cases and review of the literature. Turk Patoloji Derg 2019;35:61-8.  Back to cited text no. 2
    
3.
Bhutani N, Kajal P, Sharma U. Many faces of Wilms tumor: Recent advances and future directions. Ann Med Surg 2021;64:102202.  Back to cited text no. 3
    

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Correspondence Address:
Ankita Soni,
Department of Pathology, Dayanand Medical College and Hospital, Tagore Nagar, Ludhiana, Punjab - 141 001
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_1084_21



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