Renal teratoma with duplication of cecum and appendix

Renal teratoma with duplication of cecum and appendix

Journal of Pediatric Surgery (2010) 45, 255–258 www.elsevier.com/locate/jpedsurg Renal teratoma with duplication of cecum and appendix Madhumita Muk...

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Journal of Pediatric Surgery (2010) 45, 255–258

www.elsevier.com/locate/jpedsurg

Renal teratoma with duplication of cecum and appendix Madhumita Mukhopadhyay a , Ram Mohan Shukla b , Kartik Chandra Mandal b , Biswanath Mukhopadhyay b,⁎ a

Department of Pathology, Institute of Postgraduate Medical Education and Research, Kolkata-20, West Bengal, India Department of Paediatric Surgery, Nilratan Sircar Medical College and Hospital, West Bengal 700014, India

b

Received 8 July 2009; revised 13 October 2009; accepted 20 October 2009

Key words: Mature renal teratoma; Duplication of cecum and appendix; Teratoid Wilms' tumor

Abstract Renal teratoma is a very rare condition. We report a case of mature renal teratoma in a 1month-old male infant. The patient was admitted with a right-sided abdominal mass. The mass was found to arise from the right kidney. The tumor was removed successfully, and the patient had an uneventful recovery. The tumor mass also had an appendix and cecum within the tumor, along with a normal appendix and cecum with a separate blood supply. © 2010 Elsevier Inc. All rights reserved.

Teratomas are neoplasms that arise from pluripotent cells and can differentiate along one or more embryonic germ lines [1]. Renal teratoma is an exceedingly rare condition [2]. Teratomas commonly arise in the gonads, sacrococcygeal region, brain, and retroperitoneum. The proximity of the genital ridge to the nephrogenic anlage may partly explain how germ cells could be displaced within the kidney [3]. We describe a case of mature renal teratoma arising from the right kidney with duplication of a blind ending cecum and appendix and discuss the pathology of this rare condition.

1. Case report A 1-month-old male infant (weight 3.2 kg) was admitted with a mass in the right side of the abdomen since birth.

⁎ Corresponding author. Pediatric Surgery, N R S Medical College and Hospital, West Bengal 700014, India. Tel.: +91 033 26782929 (R), +91 09433020426 (Mobile). E-mail address: [email protected] (B. Mukhopadhyay). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.10.053

There was no history of hematuria or other systemic problems. The urinary stream was good. The patient was afebrile. His blood pressure was normal. A right-sided abdominal mass measuring 10 × 8 cm was palpable. It did not cross the midline. There were no other associated anomalies. Hemogram, urinalysis, renal and liver function tests, and chest radiograph were all within normal limits. Ultrasonography of the abdomen showed a hypoechoic, multiseptated mass (11.1 cm in its long axis) arising from the right kidney. The left kidney was normal. Contrast computed tomography (CT) of the abdomen showed a soft tissue density mass with fat and fluid components arising from the right kidney. Multiple calcifications were seen within the lesion, which measured 10.3 × 10 × 10 cm (Fig. 1). There was no enlargement of local lymph nodes. Laparotomy was done through a supraumbilical transverse transperitoneal incision. The tumor originated from the right kidney and was completely excised. There was duplication of the cecum and appendix incorporated within the tumor mass. The blood supply of the duplicated appendix and cecum was from the tumor mass and not from the colonic blood vessels (Fig. 2). The right colon with a normal appendix was in an orthotopic (normal) position. Surrounding lymph nodes and other

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Fig. 1

Contrast CT of the abdomen showing a heterogeneous mass on the right side.

organs were normal. The contralateral kidney was examined and found to be normal. The patient had a smooth postoperative recovery.

Cut section of the tumor showed extensive involvement of most of the kidney by the tumor mass (Fig. 3). It also showed a “duplicated” appendix and cecum. The central area was solid and yellow in color, with other cystic areas containing clear fluid and brain-like (glial) material. The appendix measured 8 × 0.5 × 0.4 cm. Various sections of the tumor demonstrated histologic features of mature bone, neuroepithelial, fatty and fibromuscular tissue, cartilage, and skin (Fig. 4). The section from the blind ending cecum and appendix showed mucosa typical of large bowel, containing goblet cells. Overall, the histologic features were consistent with mature benign teratoma.

2. Discussion

Fig. 2 Line diagram showing the tumor mass, appendix, and cecum with its blood supply.

Primary intrarenal teratomas are very rare. In one review, only 12 such tumors have been reported in children [2]. In the same review article, the authors mentioned 8 cases of intrarenal teratomas in adults [2]. Ledo et al [4] reported renal teratoma in an infant. Wilms' tumor, retroperitoneal tumor, neuroblastoma, hydronephrosis, mesoblastic nephroma, and renal cysts must be included in the preoperative differential diagnosis. In mature teratomas,

Renal teratoma with duplication of cecum and appendix

Fig. 3

The open specimen with variegated appearance of the tumor along with the cecum and appendix.

skin with dermal appendages, bronchial structures with bronchial glands and cartilage, neuroglial tissue, and teeth are commonly present and regarded as evidence of organogenesis [5]. The tumor in our patient weighed 425 g and involved most of the kidney except the lower pole. The tumor was partially cystic and partially solid in nature. Our patient is one of the youngest reported cases and had duplication of the cecum and appendix along with a renal teratoma. We could not find a similar association previously reported in the English literature. Various congenital anomalies have been reported with renal teratoma [2,6]. Developmental anomalies increase the risk of teratoma [2]. In another review, 10 cases of teratoma were found in rare

Fig. 4

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sites [7]. There was only 1 case of renal teratoma in an adult in that series [7]. Primary carcinoid tumors in a mature teratoma of the kidney have also been reported [8,9]. A CT scan of the mass demonstrated cystic areas [3] and coarse foci of calcifications or necrosis [9]. The role of magnetic resonance imaging in such tumors is unclear. The single reported case in which magnetic resonance imaging was performed noted a heterogeneous mass with low signal intensity [10]. During pathological evaluation of a renal teratoma, the diagnosis of a teratoid Wilms' tumor must be excluded [10,11]. Classical Wilms' tumor contains histologic findings of blastemal, stromal, and epithelial cell types. The clear indication to form nonrenal organs (as seen in our

Photomicrograph of the renal teratoma showing mature cartilage, skin, and adnexa (hematoxylin and eosin ×400).

258 patient) distinguishes teratomas from nephroblastoma [1]. An accurate histologic diagnosis is very important. Complete excision of the tumor mass is recommended. The most common presentation of an intrarenal teratoma is an abdominal mass. Other complaints may include abdominal pain, vomiting, constipation, and hematuria [12,13]. Follow-up data after surgical removal for intrarenal mature teratomas in children are limited. Mature teratomas are usually benign, but they have the potential for malignant transformation [4]. All patients with the diagnosis of benign teratoma should undergo regular long-term follow-up examinations. In our patient (although clinically doing well and currently only 4 months post resection), the serum α-fetoprotein level was evaluated twice and found to be within normal limits.

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M. Mukhopadhyay et al. [3] Otani M, Tsujimoto S, Miura M, et al. Intrarenal mature cystic teratoma associated with renal dysplasia: case report and literature review. Pathol Int 2001;51:560-4. [4] Ledo RMA, Mosquira MCF, Monjoy VMA, et al. Renal teratoma in an infant with an abdominal mass. Original article: teratoma renal en unlactante con una masa abdominal. Rev Mex Pediatr 2006;73(1):22-5. [5] Govender D, Nteene LM, Chetty R, et al. Mature renal teratoma and a synchronous malignant neuroepithelial tumor of the ipsilateral adrenal gland. Correspondence. J Clin Pathol 2001;54:253-6. [6] Lodding P, Hugosson J, Hansson G. Primary carcinoid tumour with ossification masquerading as calyx stone in a horse shoe kidney. Scand J Urol Nephrol 1997;31:575-8. [7] Bhalla S, Masih K, Rana RS. Teratomas of rare sites: a review of ten cases. J Indian Med Assoc 1991;89:291-4. [8] Kim J, Suh K. Primary carcinoid tumor in a mature teratoma of the kidney: ultrasonographic and computed tomographic findings. J Ultrasound Med 2004;23:433-7. [9] Yoo J, Park S, Jung Lee H, et al. Primary carcinoid tumor arising in a mature teratoma of the kidney: a case report and review of the literature. Arch Pathol Lab Med 2002;126:979-81. [10] Singer AJ, Ander KH. Primary teratoma of the kidney. Urology 2001; 58:1056-7. [11] Cecchetto G, Alaggio R, Scarzetto G, et al. Teratoid Wilms' tumor: report of a unilateral case. J Pediatr Surg 2003;38:259-61. [12] Aubert J, Casmayou J, Denis P, et al. Intrarenal renal teratoma in a newborn child. Eur Urol 1978;4:306-8. [13] Yaqoob N, Ahmed Z, Jafri N, et al. Renal teratoma: a rare entity. J Pak Med Assoc 2003;53:492-3.