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Kidney displaced by giant retroperitoneal liposarcoma in HIV patient

CASE DESCRIPTION

A 56-year-old male with a history of infection of human immunodeficiency virus over ten years, was referred to our center because of intermittent epigastralgia and gradual increase of abdominal girth in the last two months. Physical examination revealed palpable abdominal mass at the right upper quadrant measuring around 20cm. Laboratory examinations of complete blood counts, urine tests, and tumor markers were otherwise normal. CT scan of the abdomen showed a huge fatty mass of 23.3 x 22.9 x 34.5cm with mixed density and pathological contrast enhancement arising in the retroperitoneum. The mass displaced right kidney in epigastrium ( Figure -1A ) and most of the bowel away from their natural position in right side of abdomen ( Figure-1B ). Surgical excision of the mass was performed through a para-midline incision, and revealed a giant clearly encapsulated fatty tumor deriving from the right retroperitoneal fatty tissue ( Figure-2A ). The mass was completely extirpated without resection of adjacent tissue or organs. The final histopathological report showed a well-differentiated liposarcoma of the retroperitoneum ( Figure-2B ). The patient’s postoperative course was uneventful and he was discharged on the 6th postoperative day. At one year post-surgery, there was no evidence of recurrence on different CT scans.

Figure 1
A) Right kidney dislocated in epigastrium by the retroperitoneal component of the mass. B) Right colon displaced against abdominal wall and most of the small bowel in left side of abdomen.

Figure 2
A) Intraoperative image of the resected specimen. B) Histological examination showed presence of atypical, hyperchromatic stromal cells with a varying number of lipoblasts.

Retroperitoneum is the primary site in about 15% of soft tissue sarcomas (STS) ( 11. P.W. Pisters, M. Weiss, R. Maki, Soft-tissue sarcomas, in: D.G. Haller, L.D. Wagman, C. Camphausen, W.J. Hoskins (Eds.), Cancer Management: AMultidisciplinary Approach Medical, Surgical, & Radiation Oncology, 4th ed.,UBM Medica LLC, Norwalk, CT, 2011. ). Liposarcomas account for approximately 40% of retroperitoneal sarcomas making them the most common type ( 22. Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Curr Opin Oncol. 201l;23:373-8. ). The differential diagnoses of masses with retroperitoneal fat content is an usual diagnostic predicament. Computed tomography (CT) imaging features that suggest malignancy include large lesion size, presence of thick septa, presence of nodular and/or globular or non-adipose mass-like areas, and decreased percentage of fat composition ( 33. Kransdorf MJ, Bancroft LW, Peterson JJ, Murphey MD, Foster WC, Temple HT. Imaging of fatty tumors: distinction of lipoma and well-differentiated liposarcoma. Radiology. 2002;224:99-104. ). Histopathology is central for the distinguishing workup of lipomatous tumors. In the case that lipomalike well differentiated liposarcoma may be hard to discriminate from lipoma, an immunohistochemical panel composed of MDM2 and CDK4 can be useful ( 44. Clay MR, Martinez AP, Weiss SW, Edgar MA. MDM2 Amplification in Problematic Lipomatous Tumors: Analysis of FISH Testing Criteria. Am J Surg Pathol. 2015;39:1433-9. ).

Infection with the human immunodeficiency virus (HIV) and the subsequent destruction of T4-positive helper cells are associated with the development of various malignancies.

HIV-infected patients may be at greater risk for other forms of cancer because of changes in immune surveillance. In immunodeficient populations, other than Kaposi sarcoma and other sarcoma types, only leiomyosarcoma and angiosarcoma occur disproportionately in these patients ( 55. Bhatia K, Shiels MS, Berg A, Engels EA. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review. Curr Opin Oncol. 2012;24:537-46. ). Liposarcomas is usually a malignancy of later life but rare to be encountered in HIV populations. Although it is required to obtain negative resection margins ( 66. Vijay A, Ram L. Retroperitoneal liposarcoma: a comprehensive review. Am J Clin Oncol. 2015;38:213-9. ), it is necessary to weigh the benefit of free margin resection against the adversity of medical complication in cases where the tumor invades into a nearby organ.

REFERENCES

  • 1
    P.W. Pisters, M. Weiss, R. Maki, Soft-tissue sarcomas, in: D.G. Haller, L.D. Wagman, C. Camphausen, W.J. Hoskins (Eds.), Cancer Management: AMultidisciplinary Approach Medical, Surgical, & Radiation Oncology, 4th ed.,UBM Medica LLC, Norwalk, CT, 2011.
  • 2
    Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Curr Opin Oncol. 201l;23:373-8.
  • 3
    Kransdorf MJ, Bancroft LW, Peterson JJ, Murphey MD, Foster WC, Temple HT. Imaging of fatty tumors: distinction of lipoma and well-differentiated liposarcoma. Radiology. 2002;224:99-104.
  • 4
    Clay MR, Martinez AP, Weiss SW, Edgar MA. MDM2 Amplification in Problematic Lipomatous Tumors: Analysis of FISH Testing Criteria. Am J Surg Pathol. 2015;39:1433-9.
  • 5
    Bhatia K, Shiels MS, Berg A, Engels EA. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review. Curr Opin Oncol. 2012;24:537-46.
  • 6
    Vijay A, Ram L. Retroperitoneal liposarcoma: a comprehensive review. Am J Clin Oncol. 2015;38:213-9.

Publication Dates

  • Publication in this collection
    01 June 2020
  • Date of issue
    Jul-Aug 2020

History

  • Received
    08 Aug 2019
  • Accepted
    20 Nov 2019
  • Published
    25 Mar 2020
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