Clinicopathological Conference

1. Clinicopathological Conference Department of Surgery Aclan.Agbanlog.Agoncillo.Alianza Ame.Ancheta.Ang Ping. Ang A. Ang,J. Ang,V. Arguelles

2. Identifying Data • 52 y/o Female, Filipino, Married, from Cainta, Rizal • Admitted for the 1st time: June 20, 2010

3. Chief Complaint • Right posterolateral thigh mass of 1 year duration • Weakness of 1 week duration

4. HPI • 1 year PTA – initial symptoms • Soft, nontender, non erythematous, raised, movable, 1.5 cm posterior thigh, progressive growth • Pertinent positives: • Pertinent negatives: no bloody discharge

5. HPI • 2 months PTA- • 3 cm , inc in size, bloody discharge on manipulation • Pertinent negatives: no fever, wt loss, anorexia, nausea, vomiting, pain, limitation on movement

6. HPI • 1 week PTA • Generalized weakness, anorexia, inc in size with excessive bloody discharge (daily) • Incision & Drainage done

7. Pertinent Negatives • (-) Hyptertension, DM • (-) Past hospitalization, surgery • (-) Smoking, alcohol intake, drug abuse • (-) Family History of HTN, DM, CA

8. Pertinent Negatives • (-) Weight loss • (-) Limitation in movement • (-) Pain • (-) Exposure to radiation

9. Pertinent Positives • (+) Anorexia • (+) Bleeding, ulcerating lesion

10. Notes upon Admission • - ECOG • - Karnofsky • - pale conjunctiva, lips • - pale dry skin • - post. Lateral thigh mass • - 10x10 cm • - firm • - non movable • - pruritic on manipulation • - poorly defined borders • - Excoriating pain, necrotic • - anorexia

11. Diagnostic Work-up

12. Diagnostic Work-up

13. Diagnostic Work-up

14. Diagnostic Work-up

15. Diagnostic Work-up • CXR and EKG are normal • Wound specimen revealed heavy growth of P. mirabilis mixed with P. aeruginosa

16. Diagnostic Work-up • CT Scan (6/22/10): • An irregular mass-like density (2.0 x 4.3 x 4.6 cm) with central air density was seen on subcutaneous region of the right posterolateral thigh surrounded with fat stranding. A nodular, soft density (0.9 x 1.1 x 0.9 cm), most likely an enlarged lymph node, identified in the right inguinal region. No abnormal findings in osseous and soft tissue structures of the left thigh.

17. Problem #1Right posterolateral thigh mass

18. Problem #2Anemia & Unstable Angina

19. Problem #3Infection

20. Differential Diagnoses • Dermatofibrosarcoma Protuberans • Liposarcoma • Malignant Fibrous Histiocytoma

21. Dermatofibrosarcoma Protuberans • HISTORY AND PE • Primary fibrosarcoma of the skin • Incidence: 5% (relatively uncommon) • Age of incidence: 20-50 y/o • Rare in very young or very old • Slight male predominance • Locally aggressive • High recurrence rate

22. Dermatofibrosarcoma Protuberans • HISTORY AND PE • Presentation: Aggregated protuberant tumors within a firm indurated plaque that may ulcerate • Mobile on palpation • Bloody in latter stages • Varying color from fleshy to reddish brown

23. Dermatofibrosarcoma Protuberans • RADIOLOGIC FINDINGS • CT: Attached to the skin; used to visualize bone invasion

24. Dermatofibrosarcoma Protuberans • DIAGNOSTIC TESTS • Biopsy • Expected findings: Cellular neoplasm, composed of fibroblasts arranged radially, in a storiform pattern; Mitoses may be present; Epidermis is thinned

25. Liposarcoma • HISTORY AND PE • Old age; Mean age of incidence: 40-60 y/o • Peak incidence during 50’s • 2nd most common soft tissue sarcoma • Incidence: 14% • Male predilection • Mass is painful in 5% of patients

26. Liposarcoma • HISTORY AND PE • Presentation: slowly enlarging, painless, non-ulcerating mass • May be retroperitoneal • 40% occuring in lower extremities • Popliteal, thigh, or gluteal areas • Most patients are asymptomatic until tumor is large

27. Liposarcoma • RADIOLOGIC FINDINGS • X-ray: radio opaque • CT: indistinguishable from other soft tissue sarcomas such as MFH, dermotofibrosarcoma protuberans, etc. • MRI: may appear cystic; not preferred

28. Liposarcoma • DIAGNOSTIC TESTS • Depends on biopsy • Expected findings: lipoblasts are almost always present  indicate fatty differentiation; they mimic fetal fat cells and contain round, clear cytoplasmic vacuoles that scallop the nucleus

29. Liposarcoma • RADIOLOGIC FINDINGS • X-ray: radio opaque • CT: indistinguishable from other soft tissue sarcomas such as MFH, dermotofibrosarcoma protuberans, etc. • MRI: not preferred

30. Malignant Fibrous Histiocytoma • HISTORY AND PE • Old age; mean age of occurrence: 50-70 y/o • Most common soft tissue sarcoma • Incidence: 24% • Presentation: Enlarging, painless mass in the thigh • Typically 5-10 cm in diameter • Occurs in deep fascia or skeletal muscle • 75% occurring in lower extremities

31. Malignant Fibrous Histiocytoma • RADIOLOGIC FINDINGS • CT: nonspecific; lobulated; soft tissue; same radiodensity as muscle; • Permeative and lytic, often extending into adjacent soft tissue • if with bone involvement, parallel with that of the long bone • if subcutaneous involvement – continuous with the skin; ill defined borders • fat attenuation is not found in the tumor

32. Malignant Fibrous Histiocytoma • RADIOLOGIC FINDINGS • X-ray: soft tissue mass density • 10% will show diffuse calcifications • MRI – appears with same density as muscle

33. Malignant Fibrous Histiocytoma • DIAGNOSTIC TESTS • Needs core biopsy • Expected findings: background of spindled fibroblasts arranged in a storiform pattern admixed wit large, ovoid, bizarre multinucleated tumor giant cells

34. Clinical Impression • Soft tissue sarcoma • To Consider: • Malignant Fibrous Histiocytoma • Liposarcoma