|Year : 2014 | Volume
| Issue : 2 | Page : 121-123
A rare case of primary anterior chest wall pleomorphic liposarcoma
Prithwijit Ghosh1, Kaushik Saha2
1 Department of Pathology, DESUN (NEON) Reference Lab, Kolkata, India
2 Department of Pathology, Murshidabad Medical College and Hospital, Berhampore, Murshidabad, West Bengal, India
|Date of Web Publication||20-Jun-2014|
42/9/2, Sashi Bhusan Neogi Garden Lane, Baranagar, Kolkata - 700 036, West Bengal
Source of Support: None, Conflict of Interest: None
Primary pleomorphic liposarcoma of the chest wall or mediastinum is rare. Most of the reported cases of the chest wall liposarcomas were low-grade well-differentiated liposarcoma and/or developed from a subcutaneous lipoma. We report a very unique and unusual case of primary pleomorphic liposarcoma of anterior chest wall entrapping the anterior wall of the thoracic cage with extension into the anterior mediastinum in a 46-year-old male patient.
Keywords: Chest wall, liposarcoma, mediastinum, pleomorphic, primary
|How to cite this article:|
Ghosh P, Saha K. A rare case of primary anterior chest wall pleomorphic liposarcoma. Saudi J Health Sci 2014;3:121-3
| Introduction|| |
Primary malignant soft-tissue sarcoma of the chest wall is a rare entity, and data concerning treatment and results are sparse. Liposarcoma constitutes the 2 nd most common soft-tissue sarcoma arising in the chest wall.  Primary mediastinal liposarcomas are also rare, and are thought to represent <1% of all mediastinal tumors.  Further, pleomorphic liposarcoma is the rarest subtype of liposarcoma accounting for about 5% all liposarcomas that mostly arise in the extremities and retroperitoneum in the adult age group. , Most of reported cases of the chest wall liposarcomas were low-grade well-differentiated liposarcoma and/or developed from a subcutaneous lipoma. ,,, We present a very unique case of primary pleomorphic liposarcoma of anterior chest wall entrapping the anterior wall of the thoracic cage with extension into the anterior mediastinum.
| Case report|| |
A 46-year-old smoker and hypertensive male presented with a gradually enlarging, painless huge mass over the upper part of the left anterior chest wall for about 2 years with progressive dyspnea, myalgia, fatigue, and weight loss, after being referred from a rural hospital. He was a farmer by profession and had a remote past history of tuberculosis. On clinical examination, the mass was soft to firm, fixed to the chest wall with restricted mobility and the overlying skin was stretched, with visible venous prominence. The mass did not extend to the left axilla or back. Computed tomography (CT) scan revealed a large soft-tissue mass with heterogeneous echogenicity having its larger part bulging outside the thoracic cage with erosion of rib margins and spanning the area from 2 nd to 5 th ribs and a relatively smaller intrathoracic extrapulmonary component extending into the anterosuperior mediastinum without invasion of the lung and great vessels [Figure 1]a and b. No enlarged mediastinal lymph node was detected. CT guided fine-needle aspiration cytology (FNAC) of the mass comprised of moderately cellular small tissue fragments having atypical spindle cells and pleomorphic uni- and multi-vacuolated lipoblasts along with traversing capillaries [Figure 1]c. However, no myxoid material or chicken-wire type of blood vessel was appreciated in the smear. An 18-Fludeoxyglucose positron emission tomography/CT scan failed to demonstrate any site of metastasis. Pre-operative examinations including routine blood tests, serum biochemical assays, respiratory functions and electrocardiography were otherwise normal. En-bloc resection of the mass together with the adjacent chest wall was performed and the defect was reconstructed with a polytetrafluoroethylene mesh and latissimus dorsi muscle flap. Imprint cytology smears taken during frozen-section study of intraoperative specimen demonstrated mostly vacuolated cells mimicking lipoblasts with traversing capillaries indicating its lipomatous origin [Figure 1]d. Grossly, the resected mass was yellow, lobulated and partially encapsulated with areas of hemorrhage and necrosis [Figure 2]a. The entire tumor measured 16.5 × 12.2 × 11.8 cm and weighed 2200 g in total. Histopathological examination revealed areas mostly constituting sheets of atypical uni- and multi-vacuolated lipoblasts [Figure 2]b with hyperchromatic scalloped nuclei dissecting through the anterior chest wall muscles [Figure 2]c and small fascicles of pleomorphic spindle cells [Figure 2]d in a lipomatous background. Brisk mitotic activity and areas of necrosis were also noted. Corroborative to the FNAC findings, myxoid areas were not detected even after extensive sampling of the tumor mass. The patient's post-operative course was unremarkable, and he did not receive any adjuvant therapy. The patient was asymptomatic with no evidence of recurrence or metastasis on follow-up after 6 months of surgical resection.
|Figure 1: (a) Axial view of computed tomography (CT) scan of the chest (mediastinal window) showing a soft-tissue mass over left anterior chest wall extending into the anterior mediastinum, (b) coronal view of CT scan of the chest (mediastinal window) showing a softtissue mass over anterior chest wall extending into the anterosuperior mediastinum, (c) cytology smear showing atypical spindle cells (solid arrow), pleomorphic lipoblasts (broken arrow) and traversing capillaries (Pap, ×400), (d) photomicrograph of imprint smear prepared from the resected chest wall mass (Giemsa, ×400)|
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|Figure 2: (a) Photograph of portion of the resected chest wall mass, (b) Photomicrograph of histopathology section showing pleomorphic lipoblasts, atypical stromal cells and increased mitotic figures (H and E, ×400), (c) Photomicrograph showing anterior chest wall muscle infi ltration by the malignant cells (H and E, ×400), (d) Photomicrograph showing atypical spindle cells, bizarre looking cells and high mitotic count in a lipomatous background (H and E, ×400)|
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| Discussion|| |
Although liposarcoma is the single most common type of sarcoma occurring mostly in the adult life, it is reportedly a rare entity in chest wall as well as in mediastinum. , Most of the chest wall and mediastinal cases are well-differentiated liposarcomas and pleomorphic subtype was infrequently reported. ,,,
Fernαndez et al. presented a case of giant ulcerated pleomorphic liposarcoma of chest wall and Sezer et al.  have reported a case of pleomorphic liposarcoma arising in the pectoralis major muscle but both cases did not involve the underlying mediastinum. Shoji et al.  described a case that was to some extent similar to ours, in chest wall being the primary site of occurrence with mediastinal encroachment, but differed in being a well-differentiated subtype, with a preceding history of operated subcutaneous lipoma and significant involvement of the mediastinal structures.
On CT scans, the appearance of liposarcomas varies from a predominantly fat-containing lesion to a solid mass. Heterogeneity is related to the extent of necrosis, calcification, vascularity, cystic change, and soft-tissue component of liposarcomas. However, a pre-operative radiological diagnosis to distinguish liposarcoma from a lipoma is very difficult and histopathological diagnosis with subtyping is a must. 
As most of the primary chest wall soft-tissue sarcomas are low-grade, complete surgical excision is the preferred therapeutic choice. However, adjuvant therapy might be considered with high-grade tumors having metastasis and/or recurrence. Survival of patients with primary soft-tissue sarcomas of the chest wall after resection is similar to that of patients with sarcomas of the extremities. , In the present case, due to lack of metastasis both pre- and post-operatively during follow-up, adjuvant therapy was not considered.
Overall, prognosis correlates with the tumor subtype, as rapid tumor recurrence and metastatic disease are more common in patients with dedifferentiated and pleomorphic subtypes, whereas patients with well-differentiated tumors respond relatively well during follow-up period. Other factors that influence survival are the location and size of the tumor, and the completeness and accuracy of the surgical excision. ,,, Our case was thus unique in being a pleomorphic subtype of liposarcoma, arising primarily in the anterior chest wall, but without noticeable involvement of mediastinal structures or distant metastasis in spite of having a higher-grade.
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[Figure 1], [Figure 2]