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Year : 2019  |  Volume : 5  |  Issue : 3  |  Page : 139-141

Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation

1 Consultant Patholgist, Kovai Medical Center, Hospital, Coimbatore, Tamil Nadu, India
2 Surgical Oncologist, Kovai Medical Center, Hospital, Coimbatore, Tamil Nadu, India

Date of Submission18-Jan-2019
Date of Decision01-Sep-2019
Date of Acceptance21-Sep-2019
Date of Web Publication17-Dec-2019

Correspondence Address:
Dr, MD Rajeshwari K Muthusamy
Consultant Pathologist, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, 641014
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mamcjms.mamcjms_8_19

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Background: Paraneoplastic syndrome is a clinical syndrome with systemic effects, triggered by altered immune response to a neoplasm. Paraneoplastic syndrome was described mostly in association with epithelial malignancies, while in a soft tissue sarcoma it is unusual and hyperleucocytosis as a paraneoplastic phenomenon is very rare. Case presentation: We report a middle aged male patient with bilateral retroperitoneal masses with histomorphology of myxoid liposarcoma with dedifferentiation. Hyper-leucocytosis in the peripheral blood and pronounced myeloid hyperplasia in marrow was found with no obvious focus of infection. There was gradual reduction in white cell count in the postoperative period and reached normal values on Day 8. Hence a diagnosis of dedifferentiated liposarcoma of the retroperitoneum with hyperleucocytosis as a paraneoplastic manifestation was made. Conclusion: Paraneoplastic manifestation could probably be due to production of G-CSF by tumor cells as the leucocytosis disappeared after tumor resection. Further, leucocytosis is not reported in association with well differentiated liposarcoma, and if happens to be so, it might be a clue to search for the dedifferentiated component.

Keywords: Dedifferentiated, hyperleukocytosis, liposarcoma, paraneoplastic, retroperitoneum

How to cite this article:
Mehta SS, Muthusamy RK, Rajan F, P. S. S. Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation. MAMC J Med Sci 2019;5:139-41

How to cite this URL:
Mehta SS, Muthusamy RK, Rajan F, P. S. S. Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation. MAMC J Med Sci [serial online] 2019 [cited 2020 Apr 6];5:139-41. Available from: http://www.mamcjms.in/text.asp?2019/5/3/139/273292

  Case Report Top

A middle-aged man presented with abdominal fullness and pain of 6-month duration. On clinical examination, there was a retroperitoneal mass without any organomegaly or lymphadenopathy. Positron emission tomography–computed tomography revealed intensely hypermetabolic soft tissue mass in the retroperitoneum on the left measuring 18.6 × 10 × 9.5 cm in size and a moderately hypermetabolic heterogeneously enhancing hypodense lesion on the right side measuring 13.5 × 10 × 8.5 cm in size. There was diffuse bone marrow (BM) uptake in the entire skeleton [Figure 1]a. Complete blood count revealed leukocytosis of 75,000/mm3 with 95% neutrophils. The patient was afebrile and there was no identifiable focus of infection. BM aspirate and trephine biopsy was performed to rule out primary marrow pathology; both revealed pronounced myeloid hyperplasia with neutrophilia without increase in blasts [[Figure 1]b and 1c). There was no focus of infection and Janus Kinase 2 (JAK2 mutation) was negative. Trucut biopsy of the mass revealed a spindle cell tumor within myxoid stroma and occasional vacuolated cells resembling lipoblasts with the provisional diagnosis of liposarcoma.
Figure 1 (a) Positron emission tomography–computed tomography showing retroperitoneal masses with uptake and diffuse BM uptake. (b) BM biopsy showing very cellular marrow (H&E ×10). (c) BM biopsy showing myeloid hyperplasia and neutrophil predominance (H&E ×40). (d) Gross specimen of resected tumor masses of both sides, showing characteristic cut surfaces. (e) Sheets of pleomorphic cells and multinucleate tumor giant cells (H&E ×10). (f) Multivacuolated lipoblast in the center (H&E ×40). BM, bone marrow; H&E, hematoxylin and eosin.

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Retroperitoneal masses were resected, gross examination showed large lobulated soft tissue mass on the left side, cut surface was tan yellow with large central area of necrosis, whereas the right-sided mass had predominantly myxoid surface [Figure 1]d. Histologically, left-sided tumor revealed sheets of pleomorphic spindle cells, multinucleated tumor giant cells [Figure 1]e, numerous neutrophils, macrophages, and multivacuolated lipoblasts [Figure 1]f, and large areas of tumor necrosis, focal myxoid stroma, and slender chicken wire-like capillaries. Right-sided mass showed predominantly myxoid component with stellate to spindled cells, lipoblasts, and slender capillaries. Focal dedifferentiation was also present, suggesting the histological diagnosis of classical dedifferentiated liposarcoma (DL; left side) and myxoid liposarcoma with dedifferentiation (right side). On postoperative follow-up, there was a gradual decrease in WBC count that was within normal range in a week time. Hence, the final diagnosis of DL in the retroperitoneum with hyperleucocytosis (HL) as a paraneoplastic syndrome (PNS) was considered. During further follow-up for 18 months, the tumor recurred in the left quadratus lumborum muscle at L4–L5 vertebral level measured in size of 4.8 × 4 cm; he denied for excision and lost to follow-up further.

  Discussion Top

DL accounts for most pleomorphic sarcomas in the retroperitoneum with frequent local recurrence. Dedifferentiation in a well-differentiated liposarcoma particularly in retroperitoneum is likely to be time dependent rather than site dependent and is often associated with ring or giant marker chromosomes and consistent, substantial amplification and overexpression of murine double minute 2 gene (MDM2).[1] The prognosis is predicted by its location and not by the extent of dedifferentiated component in the tumor necessitating the need for resection.

The present case had neutrophilic leukocytosis without any identifiable infective focus; negative for JAK2 and normalized white blood cell (WBC) in the postoperative period, PNS was suspected. There are few reports of leukemoid reactions in association with DL and inflammatory malignant fibrous histiocytoma (IMFH); however with review, certain cases of IMFH have been proved to be of specific lineage.[2],[3] Hence, the reported cases of MFH with leukemoid reaction could actually had a liposarcoma lineage. Leukocytosis is due to the production of granulocyte colony-stimulating factor (G-CSF) by tumor cells, as the leukocytosis disappears after tumor resection. G-CSF, a hematopoietic growth factor, responsible for leukocytosis, enhances differentiation and accelerated maturation of the myeloid precursors into mature neutrophils in the BM and its release into the PBS.[3] STAT3, a transcription factor, plays as a key mediator in G-CSF signaling and promotes proliferation, survival, invasion, and angiogenic effects of tumor cells by regulating multiple gene products. Survivin is one such regulated gene that functions in regulating tumor growth. Chakraborty and Guha[4] indicated that G-CSF activates STAT3 and STAT3-dependent surviving expression in bladder cancer cells. The association further implies that G-CSF production and dedifferentiation in liposarcoma could share the same signaling pathway. It has been associated with poor differentiation and invasiveness in epithelial tumors, but the role of G-CSF in DL is yet to be elucidated.[3] Also, leukocytosis is not reported in well-differentiated liposarcoma, and if it does it might be a clue to search for the dedifferentiated component.

Murata et al.[5] has shown strong correlation of BM-FDG (FluoroDeoxy Glucose) uptake and WBC, especially neutrophils and marrow metabolism, which is regulated by granulocytic progenitors and stimulated by hematopoietic growth factors, that is, G-CSF. Hurtado-Cordavi et al.[6] reviewed 14 studies including 16 cases of IMFH with leukocytosis, of which 50% were in retroperitoneum suggesting that the retroperitoneal tumors had a higher recurrence and poorer prognosis with overall decreased survival.[6]To conclude, the present case of DL with leukocytosis seemed to be due to production of G-CSF by tumor cells, as the WBC count returned to normal range within a week after tumor resection. This explains the direct association between DL and leukocytosis as a PNS.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Dei Tos AP, Marino-Enriquez A, Pedeutour F, Rossi S. Dedifferentiated liposarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO Classification of Tumours of Soft tissue and Bone. 4th ed. IARC: Lyon; 2013. pp. 37-8.  Back to cited text no. 1
Nasser SM, Choudry UH, Nielsen GP, Ott MJ. A leukemoid reaction in a patient with a dedifferentiated liposarcoma. Surgery 2001;129:765-7.  Back to cited text no. 2
Sakamoto A, Matono H, Yoshida T, Tanaka K, Matsuda S, Oda Y et al. Dedifferentiated liposarcoma with leukocytosis. A case report of G-CSF producing soft tissue tumors, possible association with undifferentiated liposarcoma lineage. World J Surg Oncol 2007;5:131.  Back to cited text no. 3
Chakraborty A, Guha S. Granulocyte colony-stimulating factor/granulocyte colony-stimulating factor receptor biological axis promotes survival and growth of bladder cancer cells. Urology 2007;69:1210-5.  Back to cited text no. 4
Murata Y, Kubota K, Yukihiro M, Ito K, Watanabe H, Shibuya H. Correlations between 18F-FDG uptake by bone marrow and hematological parameters: measurements by PET/CT. Nucl Med Biol 2006;33:999-1004.  Back to cited text no. 5
Hurtado-Cordavi J, Pathak P, Avezbakiyev B, Frieri M. Inflammatory malignant fibrous histiocytoma associated with leukemoid reaction or leukocytosis: a comprehensive review. ISRN Oncol 2012;2012:946019.  Back to cited text no. 6


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