Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 33  |  Issue : 1  |  Page : 28-31

Literature review of an adult woman with Wilms' tumor


1 Department of Urology, Hisar Intercontinental Hospital, Istanbul, Turkey
2 Department of Urology, Hisar Intercontinental Hospital; Nişantaşı University, Istanbul, Turkey
3 Department of Oncology, Hisar Intercontinental Hospital, Istanbul, Turkey
4 Department of Radiology, Hisar Intercontinental Hospital; Department of Medical Imaging, Galata University, Istanbul, Turkey
5 Department of Pathology, Hisar Intercontinental Hospital, Istanbul, Turkey

Date of Submission26-Jun-2021
Date of Decision07-Jul-2021
Date of Acceptance08-Jul-2021
Date of Web Publication15-Feb-2022

Correspondence Address:
Basri Cakiroglu
Saray Mh. Siteyolu Cd. No. 7, 34768, Ümraniye, İstanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HUAJ.HUAJ_27_21

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  Abstract 


Wilms' tumor (WT), also known as nephroblastoma, is considered as an embryonal tumor due to nephrogenesis and histologic mimics of the early-onset age. WT is the most common renal tumor in children, but it is extremely rare in adults. WT is known to be a very chemosensitive tumor, and modern clinical trials aim to improve risk classification to reduce the burden of treatment. Diagnosis of WT is usually made after nephrectomy, so the possibility of preoperative chemotherapy is only possible in patients diagnosed with biopsy. A 48-year-old female with a history of 3-week left upper quadrant and left flank pain applied to a general practitioner. There was no previous trauma, hematuria, or other systemic symptoms. Further investigation with computed tomography (CT) scan of the abdomen with intravenous contrast revealed enlarged left kidney mass lesion within the left lower pole measuring up to 7.4 cm × 7.5 cm × 9.2 cm [Figure 1]. The patient underwent open radical left nephrectomy, retroperitoneal lymph node dissection, and partial excision [Figure 2]. Radical nephrectomy was performed, and when nephroblastoma was detected in histopathological examination, she was consulted with medical oncology. After positron emission tomography-CT examination revealed a thyroid involvement, a biopsy was performed, and it was diagnosed as papillary thyroid carcinoma. Total thyroidectomy was performed by the ear, nose, and throat and papillary thyroid carcinoma was diagnosed. Then, the patient started on vincristine dactinomycin therapy. There was no recurrence at the 3rd, 6th, 12th, and 36th month controls of the patient.

Keywords: Adult, nephroblastoma, Wilms' tumor


How to cite this article:
Cakiroglu B, Tas T, Solak M, Aksoy SH, Ates L. Literature review of an adult woman with Wilms' tumor. Hellenic Urology 2021;33:28-31

How to cite this URL:
Cakiroglu B, Tas T, Solak M, Aksoy SH, Ates L. Literature review of an adult woman with Wilms' tumor. Hellenic Urology [serial online] 2021 [cited 2022 May 23];33:28-31. Available from: http://www.hellenicurologyjournal.com/text.asp?2021/33/1/28/337780




  Introduction Top


Wilms' tumor (WT), also known as nephroblastoma, is the most common pediatric cancer of the kidney, most often occurring in the first 5 years of life and with the highest incidence between 3 and 4 years of age.[1] Adult WTs (AWT) are extremely rare and have been presented in the literature in about 500 cases or case series to date.[2] WT is an embryonic tumor that is thought to be originated from primitive metanephric blastemal.[1] Histologically, it is consists of three-phase model including epithelial, stromal, and blastemal components with varying degrees of expression. Histopathologically, AWT is not different from the pediatric group.[3]


  Case Report Top


A 48-year-old female, 172 cm tall and 68 kg in weight, presented with weakness, fatigue, weight loss, and abdominal distension for 3 weeks. In her examination, her blood pressure revealed 130/70 mmHg, heart rate revealed: 78/min, her temperature was 36.5, the patient was slightly pale in appearance, CVAT(Costa Vertebra Angle Tenderness) −/left+ (CVAH−/left+), she was presented with tenderness in the left upper quadrant.

There was no previous trauma and hematuria. She has been treated for 4 years due to severe depression. She had no history of other systemic disease. There was no family history of medical problems or cancer. She was a nonsmoker, had no allergies, and was not taking any regular medications.

Infection parameters were found normal in blood tests (C-reactive protein 48 mg/L, leukocytes 6.79 × 109/L) with signs of anemia (hemoglobin 11.3 g/dL, hematocrit 33.3%, and thrombocytes 285 × 109/L). Kidney functions were normal (creatinine 0.64 mg/dL, blood urea nitrogen 11.6 mg/mg/dL). Renal ultrasound revealed a hyperechoic mass measuring 9 cm in the lower pole of the left kidney. Further investigation with computed tomography (CT) scan of the abdomen with intravenous contrast revealed enlarged left kidney mass lesion within the left lower pole measuring up to 7.4 cm × 7.5 cm × 9.2 cm [Figure 1].
Figure 1: Computed tomography scan – axial view. Left kidney a mass lesion within the left lower pole measuring up to 7.4 cm × 7.5 cm × 9.2 cm

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At the end of the examinations, the patient was diagnosed with renal cancer, and open radical left nephrectomy and retroperitoneal lymph node dissection were performed. Macroscopically, nephrectomy material sized 14 cm × 9.5 cm × 7.5 cm diameter. On the cross-sectional surface, a 9 cm × 8 cm tumor showing hemorrhage and cystic areas, with solid and soft components were demonstrated [Figure 2].
Figure 2: Macroscopical nephrectomy material sized 14 cm × 9.5 cm × 7.5 cm

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Tumoral capsule invasion was detected. There was no invasion in the pelvis. In addition, the sample from the surreal tissue was 6 cm × 2 cm × 0.7 cm diameter. Microscopically, a 9 cm diameter malignant tumor formation consisting of round-oval tubular structures formed by cells with hyperchromatic nuclei, rosette structures, cord structures, papillary structures, and solid areas were observed [Figure 3]. Vascular invasion, necrosis, and hemorrhage were observed in the tumor. Capsule invasion and extracapsular soft-tissue invasion were demonstrated, although the tumor penetrated the gerota, it did not exceed to perirenal soft tissues. There was no invasion in the renal hilus either.
Figure 3: Blastemal area with tubular and small papillary structures (H and E, ×10)

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There was an invasion in the pelvis. Adrenal gland invasion was not detected. Surgical margins were tumor free. Histologically, the tumor consisted of blastemal, stromal, and anaplastic elements. Blastemal elements were present in nodular form and there was a syncytial pattern without diffuse infiltrative components. Anaplastic elements and stromal elements (nucleus three times bigger than tumor cells and tripolar mitosis) were demonstrated. Tumor tissue was consisting of round and oval cells, forming cords, and clusters. Furthermore, glomeruloid structures and focal papillary structures were demonstrated.

Fluorescent in situ hybridization analysis showed no loss of heterogeneity for 1p 16q, which was associated with poor outcomes in pediatric WT. Pathological staging was pT3a, pN0, and cM0 (pT3a – tumor invading renal capsule, but not beyond Gerota's fascia, pN0 – no metastasis in the regional lymph node, cM0 – not specified).

The patient was consulted with medical oncology. After positron emission tomography-CT examination revealed a thyroid involvement, a biopsy was performed, and it was diagnosed as papillary thyroid carcinoma. Total thyroidectomy was performed by the ear, nose, and throat. Group UH-1 protocol with early radiotherapy to the tumor bed and multiagent chemotherapy including actinomycin D, vincristine, and doxorubicin was applied to the patient. Radiotherapy and chemotherapy were started concomitantly within 3 weeks after surgery. The intensity of chemotherapy was maintained according to the protocol with granulocyte support. The patient was followed at regular intervals with CT scan and routine blood tests.

The findings of the patient's 3rd, 6th, 12th, 24th, and 36th-month control images were compared to each other and there were no signs of compatible with recurrence or metastases.


  Discussion Top


Although WT is the most common renal tumor accounting for 85% of renal neoplasms in children, it is extremely rare in adults, and <500 cases have been reported in the literature.[1],[2] WT is the most common kidney cancer in childhood with an annual incidence of 8–10/million.[4] WT has been reported as only 3% of kidney tumors in adults, which explains the difficulties in the diagnostic procedure and management of the treatment strategies in this age group.[5]

WT is characterized histologically by three types of cells: it consists of stromal, epithelial, and blastemal cells. In adults, blastemal component, which is possibly the most malignant component, is predominant. It is rare to find three patterns of cells at the same time.[6],[7] In a study conducted by the European Cancer Registry between 1983 and 1994, they found that the average age of AWT patients at the time of diagnosis was 34.[8]

Clinical and radiographic findings of WT in adults are indistinguishable from kidney tumors such as renal cell carcinoma. As a result, correct identification is usually not made until after nephrectomy, thus eliminating the possibility of neoadjuvant chemotherapy.[9] In our patient, concerning clinical and radiological findings, we concluded that the patient may have a kidney cancer and performed nephrectomy directly without biopsy. Yet, biopsy material revealed WT.

The clinical course of adults with WTs is different from that of children. While pediatric patients usually present with a painless abdominal mass, adults complain of flank, and abdominal pain, but few of them have symptoms. Some patients may present with complaints of hematuria, chest pain, back pain, abdominal mass, and hypertension. Adult patients may also present with other nonspecific symptoms such as weight loss, loss of appetite, and sudden decrease in performance.[5],[9] Our patient also presented with the complaints of weakness, fatigue, weight loss, and abdominal bloating.

Metastasis is more common in adults than in children. It is most common in the lungs, followed by the liver, bone, lymph nodes, skin, orbital, and contralateral kidney.

A wide variety of syndromes, congenital abnormalities, and structural chromosomal abnormalities have been associated with an increased risk of WT in children. Although one case of an AWT patient with a germline WT1 mutation and another with hypospadias and cryptorchidism has been identified, these syndromes do not appear to be responsible for WT in adulthood. The genetic changes that underlie WT in children contain approximately 40 cancer genes.[10]

AWT is a treatable disease. Chemotherapy is an effective treatment method, together with surgery and radiation therapy.[1]

Pediatric WT treatment is complex in itself and managed by Children's Oncology Group (COG) and International Society of Pediatric Oncology (SIOP) protocols. The two main approaches for the treatment of WTs in pediatric patients belong to the Pediatric Oncology Group and SIOP. One of the main differences between the two is that the COG approach advocates anterior nephrectomy, SIOP recommends a 4–6-week period of neoadjuvant chemotherapy for all patients older than 6 months, which allows to phase down and prevent intraoperative tumor shedding.[11]

In a study of 27 patients, four patients were Stage III, as in our patient, one of these patients received AMD + VCR, two of them AMD + VCR + ADR, and the remaining patient received postoperative chemotherapy with AMD + VCR + ADR + CPM. Three patients were treated with postoperative radiation therapy: one patient was given full abdominal radiation therapy at 4440 cGy to the tumor bed and the other two at 1080 and 1100 cGy doses, respectively. Group UH-1 protocol including radiotherapy was applied. Our patient responded objectively to this treatment. It has been stated that the role of actinomycin D in the long-term survival of patients is also unclear.

Gender is not an important prognostic factor in the pediatric age group, and only metastatic disease has been reported to have worse survival with stage-specific treatment. However, in the adult group, 5-year relative survival (61%) has been reported to be almost 30% higher in women compared to men.[8]

In a series of 27 patients, 2-year survival was reported as 67%. Depending on the tumor stage and histology, they recommend treating risk-adapted adults.[12] Our patient is now being checked for the 3rd year, and no relapse was detected during this period.


  Conclusion Top


Unlike children, WT is seen rarely in adult patients. There are no established protocols for the treatment of this age group. The diagnosis is usually made in postoperative histopathological examination.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that their name and initial 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.
Modi S, Tiang KW, Inglis P, Collins S. Adult Wilms' tumour: Case report and review of literature. J Kidney Cancer VHL 2016;3:1-7.  Back to cited text no. 1
    
2.
Ferreira E, Mohaghegh M, Venkat S, Drachenberg D, Battistuzzi S, Ji S. A case of an adult wilms tumour in a patient with velocardiofacial syndrome. Urology 2020;137:e8-9.  Back to cited text no. 2
    
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Babaian RJ, Skinner DG, Waisman J. Wilms' tumor in the adult patient: Diagnosis, management, and review of the world medical literature. Cancer 1980;45:1713-9.  Back to cited text no. 3
    
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Spreafico F, Bellani FF. Wilms' tumor: Past, present and (possibly) future. Expert Rev Anticancer Ther 2006;6:249-58.  Back to cited text no. 4
    
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Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N. Wilms' tumor in adults: Results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study. J Clin Oncol 2004;22:4500-6.  Back to cited text no. 5
    
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Caramanti RL, Aprígio RM, de Moraes DF, Rocha CE, Meguins LC, Goes MJ, et al. Brain metastasis of Wilms tumor in adult. World Neurosurg 2020;138:422-4.  Back to cited text no. 6
    
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Coppes MJ, Pritchard-Jones K. Principles of Wilms' tumor biology. Urol Clin North Am 2000;27:423-33.  Back to cited text no. 7
    
8.
Mitry E, Ciccolallo L, Coleman MP, Gatta G, Pritchard-Jones K; EUROCARE Working Group. Incidence of and survival from Wilms' tumour in adults in Europe: Data from the EUROCARE study. Eur J Cancer 2006;42:2363-8.  Back to cited text no. 8
    
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Bradtke M, Rink M, Büscheck F, Sauter G, Dahlem R, Fisch M, et al. Current therapies of wilms tumors in the adult: Diagnostic considerations and treatment challenges. Clin Genitourin Cancer 2019;17:e522-5.  Back to cited text no. 9
    
10.
Treger TD, Chowdhury T, Pritchard-Jones K, Behjati S. The genetic changes of Wilms tumour. Nat Rev Nephrol 2019;15:240-51.  Back to cited text no. 10
    
11.
Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, et al. Advances in Wilms tumor treatment and biology: Progress through international collaboration. J Clin Oncol 2015;33:2999-3007.  Back to cited text no. 11
    
12.
Arrigo S, Beckwith JB, Sharples K, D'Angio G, Haase G. Better survival after combined modality care for adults with Wilms' tumor. A report from the National Wilms' tumor study. Cancer 1990;66:827-30.  Back to cited text no. 12
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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