JOURNAL OF EVIDENCE BASED MEDICINE AND HEALTHCARE

Table of Contents

2019 Month : February Volume : 6 Issue : 5 Page : 315-316

ROUNDCELL TUMOUR OF THE KIDNEY- A RARE ENTITY.

Sravanthi Garlapati1, Vyshnavi Bollam2, Venkataram Reddy Kuchukulla3, Ramakrishnareddy Gaddam4

1. 2nd Year Resident, Department of Radiology, SVS Medical College and Hospital, Telangana.
2. 2nd Year Resident, Department of Radiology, SVS Medical College and Hospital, Telangana.
3. Professor and HOD, Department of Radiology, SVS Medical College and Hospital, Telangana.
4. Professor, Department of Radiology, SVS Medical College and Hospital, Telangana.

Corresponding Author:
Dr. Vyshnavi Bollam,
Flat No. 104, Sri Srinivasa Homes,
Vysya Bank Colony, Old Bowenpally,
Secunderabad- 500011, Hyderabad.
E-mail: docvysh@gmail.com
DOI: 10.18410/jebmh/2019/64

PRESENTATION OF CASE
A 19 years old girl came to surgery outpatient department with chief complaint of pain abdomen since one month. On physical examination there was mild tenderness in left hypochondrium. She was advised further evaluation by routine investigations and special investigations like USG and CT abdomen.

How to cite this article

Garlapati S, Bollam V, Kuchukulla VR, et al. Roundcell tumour of the kidney- a rare entity. J. Evid. Based Med. Healthc. 2019; 6(5), 315-316. DOI: 10.18410/jebmh/2019/64

PRESENTATION OF CASE

A 19 years old girl came to surgery outpatient department with chief complaint of pain abdomen since one month. On physical examination there was mild tenderness in left hypochondrium. She was advised further evaluation by routine investigations and special investigations like USG and CT abdomen.

USG Abdomen

Single well defined predominantly cystic mass lesion noted in left hypochondrium measuring approximately 12*9 cm causing mass effect on underlying left kidney, spleen and taking minimal flow on application of colour doppler.

 Image 1. USG Abdomen Showing Hypoechoic Mass Lesion in Left Hypochondrium

 

CECT Abdomen

  • Single large well-defined hypodense mass lesion predominantly cystic with multiple solid components noted occupying left hypochondrium and left lumbar region. On post contrast above mentioned mass lesion showed mild peripheral enhancement and mild enhancement of solid components with large necrotic component measuring approximately 16*16*15 cms.
  • Left adrenal gland is not visualised separately from mass lesion.
  • Rest of the kidney showed normal enhancement and excretion of contrast except for compression of upper pole.

 

 

 

Image 2. Plain CT Abdomen Coronal, Sagittal and Axial Planes Showing Mixed Dense Lesion Predominantly Hypodense in Left Hypochondrium

 

 

 

Image 3. CECT Abdomen Showing Mixed Dense Mass Lesion with Mild Peripheral Enhancement

 

 

DIFFERENTIAL DIAGNOSIS

  • Wilms Tumour
  • Neuroblastoma
  • Renal Cell Carcinoma
  • Malignant Lymphoma

 

PATHOLOGICAL DISCUSSION

Round cell tumours are rare malignancies affecting young adults,1,2 with male to female ratio 2:1 to 9:1, which is first described by Gerald and Rosai.3 These unusual tumours primarily involve abdominal serosa and only few cases are reported with kidney as a primary site.5,6

Clinical features are generally nonspecific and are diagnosed usually in later stages with wide spread metastasis but in our case patient presented with mass and pain abdomen. Ultrasonography is the initial diagnostic method of choice but if findings of ultrasonography are inconsistent then cross-sectional imaging like CT will be the next step for work up. CT with single post contrast phase i.e. portal venous phase is useful for evaluation.1

On CT heterogeneous enhancement with few hypoattenuating areas which are suggestive of necrosis fibrosis and haemorrhage are seen.1,7

Pathological analysis is definitely needed for confirmation of diagnosis.

Our histopathological diagnosis is round cell tumour but for further cell typing immunohistochemistry markers are required.

There is no specific standardized treatment, it is generally multimodal. Cases without extraperitoneal metastasis treated with pre and post-operative chemotherapy and post-operative radiotherapy, whereas cases with extra peritoneal metastasis are treated with chemotherapy alone.4

 

 

 

Image 4. Gross Specimen

 

 

Histopathology

Sections studied show tumour in sheets with intervening fibro myxoid stroma, vague rosettoid pattern with extensive areas of necrosis. Tumour cells are mildly pleomorphic, round to oval with scanty cytoplasm, nuclei with irregular contours, coarse chromatin, convoluted borders and inconspicuous nucleoli. No typical mitotic figures seen.

 

Possibilities

  • Round Cell Tumour
  • Ewing’s/PNET
  • Wilms Tumour
  • Desmoplastic Round Cell Tumour

 

 

Image 4. Histopathology Slides Showing Numerous Round Cells

 

 

DISCUSSION OF MANAGEMENT

Left radical nephrectomy done and sent for histopathological examination.

 

FINAL DIAGNOSIS

Round Cell Tumour of Kidney.

 

REFERENCES

  1. Iyer RS, Schaunaman G, Pruthi S, et al. Imaging of pediatric desmoplastic small-round-cell tumor with pathologic correlation. Curr Probl Diagn Radiol 2013;42(1):26-32.
  2. Li G, Wang HT, Gao Y, et al. Primary abdominopelvic desmoplastic small round cell tumor: CT and correlated clinic-pathologic features. Eur Rev Med Pharmacol Sci 2014;18(18):2670-2677.
  3. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989;9(2):177-183.
  4. Honoré C, Amroun K, Vilcot L, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol 2015;22(4):1073-1079.
  5. Egloff AM, Lee EY, Dillon JE, et al. Desmoplastic small round cell tumor of the kidney in a pediatric patient: sonographic and multiphase CT findings. AJR Am J Roentgenol 2005;185(5):1347-1349.
  6. Eklund MJ, Cundiff C, Shehata BM, et al. Desmoplastic small round cell tumor of the kidney with unusual imaging features. Clin Imaging 2015;39(5):904-907.
  7. Chung EM, Biko DM, Arzamendi AM, et al. Solid tumors of the peritoneum, omentum, and mesentery in children: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics 2015;35(2):521-546.