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Giant liposarcoma in scapular region: a case report.

INTRODUCTION: Liposarcomas are the second most malignancy of soft tissue, which hallmark are lipoblasts or immature fat cell. (1-3) These are usually found in late adult life and rarely in childhood with a male/female ratio of 2:1. (1,4,5) Most of the cases are de novo and frequently develops from deep seated stroma rather than sub-mucosal or subcutaneous fat. (2,6)

CASE REPORT: A 48 years male was presented to the outpatient department with chief complaint of backache due to a huge mass in upper back and foul smell. History revealed that a pea nut sized bulging was noticed by him in scapular region for 10 years and which had been increasing very slowly. When it attained the size of a cricket ball, he consulted a local surgeon, who advised him for fine needle aspiration cytology.

FNAC report primarily revealed to be an atypical lipomatous tumor; to which initially he ignored as it was not causing too much distress; but after 3 months he felt that this tumor had been increasing very rapidly; then he consulted us in our medical institute where on examination a foul smelling giant mass in the left scapular region was found (Fig-1); which was extending from mid axillary line to vertebral column and from shoulder to 12th rib and on palpation it was non-tender and firm to hard in consistency. The skin over tumor was ulcerated on the lateral side.

The CT scan showed a 60 x 45 x 35 cm mass, occupying almost left half of back, extending to anterior chest wall up to midline, inferiorly up to left renal angle and crossing mid line posteriorly (Fig-2); which was non-enhancing non-calcific, multi-compartmental soft tissue mass in relation to left scapula without obvious bony erosion; while X-ray of chest showed a huge smooth rounded opacity in the back of the chest wall without any bony involvement (Fig-3). His ultrasound report of pelvic and abdominal regions was normal. Trucut biopsy reported the mass primarily to be a low grade liposarcoma.

After thorough clinical examination; wide surgical excision of this tumor was planned under general anesthesia and hence the patient was sent for pre anesthetic evaluation where he was investigated properly. Except being thin built and low hemoglobin (Hb-9.5 gm %) his routine hematological, biochemical and serological investigations were within normal limits. Hence one unit of blood was transfused after cross matching and blood bank was informed to keep ready sufficient amount of blood.

Since the tumor was so huge that laying in supine position to this patient was not possible; hence under all vitals monitoring like ECG, SP[O.sub.2], ETC[O.sub.2], NIBP, CVP and temperature he was intubated for general anesthesia with a flexo-metallic endo-tracheal tube of 8 mm internal diameter in lateral position by our anesthesia team.

An elliptical incision was made and skin flaps were raised, and the tumor was resected in toto with preservation of the muscles, vessels & nerves and hemostasis was secured and wound was closed (Fig-4). Intraoperative period was uneventful except two unit of blood was transfused due to some blood loss. The total excised specimen of tumor was weighted 16 Kgs and was sent for histopathology. During post-operative the patient was stable.

Stitches were removed on 14th post-operative day (Fig-5). Final histopathological report revealed that the tumor had sheets of adipocytes in a uniform pattern and lipoblasts present in it which have large atypical nuclei suggestive of an adipocytic liposarcoma (Fig-6). The Patient was referred to the department of oncology for further management, where he was advised for follow up.

The patient came for first follow up after three months; found a healthy scar without any signs of local recurrence. The patient had been visiting regularly in our OPD for follow up and in last visit after passing two years of surgery; no any sign of recurrence was found.

DISCUSSION: The original description of the "lipoma-like" liposarcoma was first of all given by Enterline et al. (7,8) Liposarcomas are the most common soft tissue sarcoma; which accounts approximately 16-18% of all mesenchymal malignancies (3,9) and usually involve trunk especially retroperitoneum or abdominal cavity (42%), lower extremities (41%); apart from these they may occur at upper extremity (11%), head and neck region include the larynx, hypopharynx, oral cavity, orbit, scalp and soft tissues of the neck (6%) and more rarely in mediastinum & scrotum/spermatic cord. (1-3,6,10-16)

Considering currently available data liposarcomas are divided into several subtypes as well-differentiated, myxoid, round cell, pleomorphic, and dedifferentiated; but broadly they may be conceptualized in to two groups myxoid/round cell and well differentiated/dedifferentiated liposarcomas (1,2). Most of myxoid/round cell liposarcomas of are found primarily in the limb and their severity may vary from low grade to high-grade tumor as seen in pure myxoid and pure round cell liposarcoma respectively; but in some cases transitional features may also be seen which behavior may depend upon the presence of round cell quantity. (2,6)

The well-differentiated/dedifferentiated liposarcomas occur in elderly age as limb or retroperitoneal lesions; which contain mature fat mingle with atypical hyperchromatic cells and seldom lipoblasts; which are characterized by a worsen prognosis because of their higher recurrence rate, (17) and usually these tumor do not metastasize, (2) although their behavior depends on their histological grading and also strongly influenced by location as worse prognosis is seen in retroperitoneal liposarcomas, (2,18,19) but the role of the size of tumor in prognosis is not clear. (12)

In 2002; WHO has classified well-differentiated liposarcoma into four subtypes as adipocytic (or lipoma-like), sclerosing, inflammatory, and spindle cell liposarcomas; in which commonest is adipocytic or lipoma-like liposarcoma where proliferation of relatively mature adipocytes found (11). Sclerosing liposarcoma is the next common type and most frequently found as retroperitoneal or paratesticular lesions; and characterized by the presence of bizarre stromal cells with marked nuclear hyperchromasia and rarely multivacuolated lipoblasts mixed in fibrillary collagenous stroma; and occasionally these fibrous component may represent the most of the neoplasm. Inflammatory variety is rarely seen; which occurs most often in the retroperitoneal area; where chronic inflammatory cells are predominantly infiltrated in relatively mature adipocytes. (16)

The spindle-cell liposarcoma is quite common which usually occur in subcutaneous tissue of the upper extremities and shoulder region; where relatively bland neural appearing spindle cells mixed with fibrous and/or myxoid stroma of atypical liposarcomatous component are found (21). The myxoid subtype accounts for 50% of all liposarcomas 22; and they have predilection for the limbs.

Albeit rare, such tumors arise in the sub-cutis with involvement of dermis and are seen most often in male patients and in an acral location; and histologically they contain mature adipocytes, thick-walled blood vessels, and smooth muscle cells arranged in well-defined fascicles and around blood vessels, (23) while in pleomorphic liposarcoma CT or MR imaging shows an intense enhancement, adjacent organs invasion, vascular infiltration, calcification or ossification, and areas of necrosis or hemorrhage. (24,25)

Recently karyotypic analysis in about 80% of well-differentiated liposarcomas has shown that this group of mesenchymal neoplasm is characterized by the presence of supernumerary rings or giant marker chromosomes, or both; which contain amplified sequences derived from the chromosomal region 12q13-15; wherein they map several proto-oncogenes such as MDM2, CDK4, HMGA2, SAS, GLI, DDTI3, OS1, and OS9; which play important roles in the molecular pathogenesis of human neoplasia. According to another authors the immunostaining of CDK4 and MDM2 proteins have been recognized as an important adjunct for differentiating the benign lipoma from well-differentiated liposarcoma. (11,26-33)

CT scan of liposarcoma usually shows well-circumscribed lobulated mass with multiple septations and variable contrast enhancement and on caparison with MRI found that CT scan is superior for evaluating of cortical bone erosion and tumor mineralization. MRI findings of different types liposarcomas can be correlated with the histo-pathologic features and found that all such tumors showed well-defined margins; in which the well-differentiated liposarcomas were composed mainly of fat with septations or nodules, were hyper-intense on T2-weighted images, and demonstrated faint enhancement or no enhancement following intravenous contrast; while myxoid liposarcomas were homogeneous or mildly heterogeneous; although in some cases pseudo-capsules were also seen. (4,20)

Pleomorphic type of liposarcomas showed a markedly heterogeneous internal structure. Both myxoid and pleomorphic liposarcomas showed moderate or marked heterogeneous enhancement after contrast administration. Well-differentiated liposarcomas may be differentiated from other types of the tumor by their largely lipomatous appearance. The malignancy grade increases in parallel with tumor heterogeneity and contrast enhancement. (20,34)

The role of radiation in liposarcomas is debatable, sometimes it may be beneficial adjunct to surgery in cases where wide marginal resection of tumor was possible. According to some authors the risk of local recurrence may reduce with radiation therapy, (34-36) but others are claimed that this may aggravate dedifferentiation to a higher grade in case of recurrence. Nevertheless patient with large high grade liposarcomas may be benefited by use of multimodality treatment along with chemo-radiation; but still use of chemotherapy in liposarcomas is experimental. (37)

In conclusion, giant liposarcomas in scapular region are extremely rare; and possible differential diagnosis may be lipoma, lipoblastoma, pleomorphic lipoma, chondroid lipoma, hibernoma and neurofibroma etc. (22,36) Their choice of treatment is wide surgical excision; although with incomplete excision chances of recurrence is 17% to 80% (37) and overall mortality varies from 0% for surgical resectable soft tissue to 80% for high grade retroperitoneal liposarcomas. (11) We stress the importance of meticulous evaluation of all required investigations like biopsy, CT or MRI to see extent of lesion, prior to successful surgical and other management.

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DOI: 10.14260/jemds/2014/3196

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AUTHORS:

[1.] Krishna Gopal

[2.] Sanjeev Kumar

[3.] Sushmita

[4.] Ashwani Kumar

[5.] Ashish Prasad

[6.] S.K. Jha

[7.] K.H. Raghwendra

[8.] Sangeeta Pankaj

PARTICULARS OF CONTRIBUTORS:

[1.] Assistant Professor, Department of General Surgery, IGIMS, Patna.

[2.] Additional Professor, Department of Anaesthesiology & Critical Care Medicine, IGIMS, Patna.

[3.] Lecturer, Department of Maxillofacial & Dental Surgery, BRAIDS, Patna.

[4.] Senior Resident, Department of General Surgery, IGIMS, Patna.

[5.] Senior Resident, Department of General Surgery, IGIMS, Patna.

[6.] Professor, Department of General Surgery, IGIMS, Patna.

[7.] Professor, Department of Anaesthesiology & Critical Care Medicine, IGIMS, Patna.

[8.] Associate Professor, Department of Gynaecology Oncology, RCC, IGIMS, Patna.

NAME ADDRESS EMAIL ID OF THE CORRESPONDING AUTHOR:

Dr. Krishna Gopal, Assistant Professor, Department of General Surgery, Indira Gandhi Institute of Medical Sciences, Patna-800014, Bihar, India.

Email: gopakrishh@gmail.com

Date of Submission: 28/07/2014.

Date of Peer Review: 29/07/2014.

Date of Acceptance: 06/08/2014.

Date of Publishing: 13/08/2014.
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Title Annotation:CASE REPORT
Author:Gopal, Krishna; Kumar, Sanjeev; Sushmita; Kumar, Ashwani; Prasad, Ashish; Jha, S.K.; Raghwendra, K.H
Publication:Journal of Evolution of Medical and Dental Sciences
Date:Aug 14, 2014
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