Roberto N. Gonzales Jr. MD.

Department of Surgery

Ospital ng Maynila Medical Center

E.N.

48/F

From Tondo, Manila

CHIEF COMPLAINT:

Mass on right thigh

HISTORY OF PRESENT ILLNESS

                                                  5 mos PT    (+) pain on right thigh

                                                  (-) consult

                                                  (+) meds- mefenamic acid

3 mos PTA (+) pain

                                                (+) enlargement of   right thigh

                                                (+) Difficulty in     ambulation

1 mos PTA           Inc. severity of symptoms

                                                (+) consult PGH

                                                (+) meds- Tramadol cloxacillin

                                                no relief

2 wks PTA         - further worsening of                                                                                      symptoms,

                                                - return back to PGH

                                                - X-ray R thigh- VOS intact

                                                - CT scan was requested

- no diagnosis

1 day PTA         - consult at OMMC surgery OPD

                                                                - with CT scan request from DO - Needle aspiration

                                                                - dark red serous aspirate

                                                                PWI: Soft tissue tumor

                                   ADMISSION

Past Medical History:

                No history of previous hospitalization nor operation

                (-) DM, (-) Asthma (-) heart dse

Family History

                colon cancer – mother

Personal and Social History

Banana vendor – 2 years ago

Hog raiser- 1 year ago

Non- smoker, non- alcoholic beverage drinker

Review of System

(+) weight loss (30% in 5 mos)

(+) easy fatiguability

(+) Dizziness

(+) Dyspnea on effort

(+) loss of appetite

PHYSICAL EXAMINATION

Conscious, coherent, wheelchair borne

BP 100/70  PR 98    RR 26  T 37.9’C

SHEENT: Pale skin and palpebral conjunctiva, anicteric sclerae, no TPC, no CLAD

CHEST/LUNGS: SCE, no retractions, clear BS

HEART: adynamic precordium, NRRR, no murmur

PHYSICAL EXAMINATION

ABDOMEN:

 Soft abdomen with normoactive bowel sounds,  (-) tenderness , (-) organomegaly , (-) mass

PHYSICAL EXAMINATION

Extremities:

Left thigh – Greatest circumference

                                42 cm, essentially normal

Right thigh – enlarged, GC 90 cm,

                - more pronounced enlargement on the posterior aspect

                - hard, fixed, poorly circumscribed, non- tender

                - (+) ulcers

No inguinal lymph nodes

SALIENT FEATURES

48/M

Pain

Mass on right thigh

hard, fixed, poorly circumscribed, non- tender

 Signs & Symptoms of anemia

   easy fatiguability, Dizziness, Dyspnea on effort

 weight loss (30% in 5 mos)

PARACLINICAL DIAGNOSTIC PROCEDURE

DO I NEED A PARACLINICAL DIAGNOSTIC PROCEDURE?

                        yes

To increase the degree of certainty of my primary clinical diagnosis

To know the extent of the tumor

Paraclinicals

Biopsy

CT- Scan

PRE-TREATMENT DIAGNOSIS

PRIMARY DIAGNOSIS:

                Malignant soft tissue tumor (95%)

 Benign soft tissue tumor (5%)

PARACLINICAL DIAGNOSTIC PROCEDURE

DO I NEED ANOTHER PARACLINICAL DIAGNOSTIC PROCEDURE?

                        yes

To ascertain absence of metastasis

Paraclinicals

Chest X- Ray

Normal

PRE-TREATMENT DIAGNOSIS

PRIMARY DIAGNOSIS:

 Soft tissue Sarcoma without metastasis

                ( T2b, N0, M0 Stage III)            (95%)

SECONDARY DIAGNOSIS:

Soft tissue Sarcoma with

                                metastasis

                ( T2b, N0, M1 Stage IV)               (5%)

TREATMENT GOALS

Soft Tissue Sarcoma

Remove the lesion

Prevent recurrence

Malignant Soft-Tissue Lesion

                Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity

 The amputation group achieved significantly better local control than the limb-sparing group

No survival benefit in the groups selected for amputation when compared with patients undergoing a limb-sparing procedure with similar tumors.

 amputation can be recommended only when a limb-sparing procedure cannot achieve gross resection of tumor while still preserving a useful extremity, because amputation improves only local control and does not address distant disease.

Williard WC, Hajdu SI, Casper ES, Brennan MF.

Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.

                Association of local recurrence with subsequent survival in extremity soft tissue sarcoma

there is a strong association of local recurrence with the development of subsequent metastasis and tumor mortality, and that local recurrence is a poor prognostic factor.

It would seem prudent to consider patients who develop local recurrence and have high-grade tumors as being at high risk for systemic disease.

                Journal of Clinical Oncology, by American Society of Clinical Oncology

                JJ Lewis, D Leung, M Heslin, JM Woodruff and MF Brennan
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

Malignant Soft-Tissue Lesion

TREATMENT PLAN

                External Hemipelvectomy Right

PREPARATION and MONITORING

PSYCHOSOCIAL  SUPPORT

SCREENING FOR MEDICAL PROBLEMS:

CHEST X-RAY - normal

ECG - normal

CBC

PREPARATION and MONITORING

OPTIMIZE PHYSICAL CONDITION

Correct anemia

Adequate hydration

PROPHYLACTIC ANTIBIOTICS

PREPARATION and MONITORING

SURGICAL TREATMENT
 Operative Technique

1. Positioning the patient (lateral).

2. Ilioinguinal dissection and pelvic exploration.

3. Dissection and ligation of the internal (hypogastric)

                                iliac vessels.

4. Elevation of posterior thigh flap with external

                                iliac/superficial femoral artery.

5. Ligation of profunda femoris.

6. Osteotomy of pubic symphysis and sacroiliac joint

                                (or sacral alar).

7. Division of pelvic floor muscles.

8. Closure via flap rotation.

Peri-operative Monitoring

Total fluid given -  950 cc

Total Blood given – 2 u FWB

Estimated Blood loss – 1, 500 cc

Urine output – 550 cc

OR time ( 7am- 12:30 pm) 5 ½ hrs

OPERATION DONE

                External Hemipelvectomy Right

POSTOPERATIVE DIAGNOSIS

SURGICAL TREATMENT
POST-OPERATIVE CARE

SUPPLY THE BASIC NEEDS OF THE PATIENT

COMFORT

ANALGESICS

MEDICATIONS – ANTIBIOTICS

FLUIDS AND ELECTROLYTES

SUPPORT ORGAN FUNCTION

WOUND CARE

MONITORING FOR COMPLICATIONS

ADVICE ON

PSYCHOLOGICAL AND FUNCTIONAL REHABILITATION

HOME CARE

FOLLOW-UP PLAN

SURGICAL TREATMENT
POST-OPERATIVE CARE

RESOLUTION OF THE HEALTH PROBLEM

LIVE PATIENT

NO COMPLICATION

NO DISABILITY

SATISFIED PATIENT

NO MEDICO-LEGAL SUIT

DISCUSSION AND SHARING OF INFORMATION

Sarcomas

are a heterogeneous group of tumors that arise predominantly from the embryonic mesoderm, but also can originate, as does the peripheral nervous system, from the ectoderm.

Subtypes

Soft tissue sarcomas- largest of these groups

bone sarcomas (osteosarcomas and chondrosarcomas, Ewing's sarcomas )

Peripheral primitive neuroectodermal tumors.

Clinical Presentation

most commonly presents as an asymptomatic mass.

 The size at presentation is usually associated with the location of the tumor.

 Smaller tumors are generally located in the distal extremities,

proximal extremities and retroperitoneum can grow quite large before becoming apparent.

often grow in a centrifugal fashion and compress surrounding normal structures.

 impingement on bone or neurovascular bundles produces pain, edema, and swelling.

Differential Diagnosis

benign lesions

 lipomas,

 lymphangiomas

leiomyomas,

neuromas.

malignant lesions

primary or metastatic carcinomas

 melanomas

lymphomas

soft tissue sarcomas

annual incidence  in the United States for 2007 is estimated to be about 9,220 cases

overall mortality rate of approximately 3,560 cases per year, which includes adults and children

5-year survival rate 50-60%.

soft tissue sarcomas

account for 1% of all adult malignancies and 15% of pediatric malignancies

 External radiation therapy (RT) is a risk factor

most commonly metastasize to the lungs

tumors arising in the abdominal cavity more commonly metastasize to the liver and peritoneum.

Relative Frequency of Histologic Subtypes of Soft Tissue Sarcoma

Malignant fibrous histiocytomas -             28%

Liposarcoma-                                                                     15%

Leiomyosarcoma -                                                           12%

Unclassified sarcoma -                                   11%

Synovial sarcoma -                                                           10%

Malignant peripheral nerve sheath

                                tumor -                                                                 6%

Rhabdomyosarcoma -                                    5%

Relative Frequency of Histologic Subtypes of Soft Tissue Sarcoma

Liposarcoma-                                                                     20%

Leiomyosarcoma -                                                           19%

Malignant fibrous histiocytomas -             18%

Unclassified sarcoma -                                   22%

Fibrosarcoma -                                  10%

Synovial sarcoma -                                                             7%

Malignant peripheral nerve sheath

                                tumor -                                                                   4%

Soft tissue Sarcomas

Relative Frequency as to Location

Extremity                                                            59%

Trunk                                                                    19%

retroperitoneum                                              13%

and head and neck                            9%

2002 American Joint Committee on Cancer staging system

Tumor Size

T1 lesions are 5 cm or smaller

T 2 lesions are larger than 5 cm.

2002 American Joint Committee on Cancer staging system

Anatomic location

 above the superficial investing fascia- "a" lesions in the T score

invading or deep to the fascia as well as all retroperitoneal, mediastinal, and visceral tumors -"b" lesions.

2002 American Joint Committee on Cancer staging system

 Low grade

                                (G1) well differentiated

                                (G2) moderately differentiated

 High grade

                                (G3) poorly differentiated

                                (G4) undifferentiated

2002 American Joint Committee on Cancer staging system

French Federation of Cancer Centers system

5-year survival rates

grade 1-   90%

                                  2-   70%

                                  3-   40%

Metastatic potentials as to Tumor Grades

5 to 10% for low-grade lesions

25 to 30% for intermediate-grade lesions

50 to 60% for high-grade tumors

Practice Guidelines
in Oncology – v.1.2007
Soft Tissue Sarcoma

Consideration of Amputation

Extensive soft tissue mass and/or skin                  involvement

Involvement of a major artery or nerve

Extensive bony involvement necessitating          whole bone resection

Failure of preoperative chemotherapy or            radiation therapy

Tumor recurrence after prior adjuvant radiation

                Prognostic factors predictive of survival and local recurrence for extremity soft tissue sarcoma.

A histologic diagnosis of Ewing's sarcoma, synovial sarcoma, and angiosarcoma was associated with a 13-fold increased risk of death compared with liposarcoma, fibrosarcoma, and malignant peripheral nerve sheath histologic types after having adjusted for the other prognostic factors

                S Singer, J M Corson, R Gonin, B Labow, and T J Eberlein

                Department of Surgery, Brigham & Women's Hospital/Harvard Medical School, Boston, Massachusetts.

                Prognostic factors predictive of survival and local recurrence for extremity soft tissue sarcoma.

histologic type, high-grade sarcomas, sarcomas greater than 10 cm in size and age at diagnosis were found to be important prognostic factors for survival but not for local recurrence. 

                \

                Prognostic factors predictive of survival and local recurrence for extremity soft tissue sarcoma.

CONCLUSIONS:

   The use of mitotic activity along with grade, size, histologic type, and age at diagnosis is prognostic for survival in extremity soft tissue sarcoma.

   The use of an objective pathologic feature, such as mean mitotic activity, is also useful in selecting patients for future systemic neoadjuvant or adjuvant trials and primary therapy.

                \

                The prognostic value of histologic subtypes in primary extremity liposarcoma

The prognosis of patients with extremity liposarcoma was analyzed according to histopathologic subtypes.

A system of five subtypes and 5-year treatment failure rates and survival rates

well-differentiated- 30% and 100%

myxoid- 25% and 88%

 fibroblastic 60% and 58%

Lipoblastic- 100% and 40%

pleomorphic liposarcomas - 64% and 56%.

Chang HR, Hajdu SI, Collin C, Brennan MF.

                Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021

                The prognostic value of histologic subtypes in primary extremity liposarcoma

                .

Distant metastasis after the initial operation was not found in patients with well-differentiated liposarcoma and rare in the patients with myxoid liposarcoma.

In contrast, 50% of the patients with fibroblastic, lipoblastic, and pleomorphic liposarcoma had a distant relapse within 5 years.

Chang HR, Hajdu SI, Collin C, Brennan MF.

                Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021

                Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity

The amputation group achieved significantly better local control than the limb-sparing group

No survival benefit could be demonstrated, however, in the groups selected for amputation (i.e., large, high-grade tumors) when compared with patients undergoing a limb-sparing procedure with similar tumors.

Williard WC, Hajdu SI, Casper ES, Brennan MF.

Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.

                Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity

Prevention of local recurrence by amputation also did not improve survival in this group compared with similar patients undergoing limb-sparing surgery who did develop a local recurrence.

The prognosis of patients most likely to undergo an amputation for extremity soft tissue sarcoma (those with high-grade, large tumors) is not related to their local disease, but rather to the risk of distant metastases.

Williard WC, Hajdu SI, Casper ES, Brennan MF.

                Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.

                Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity

amputation in this cohort of patients can be recommended only when a limb-sparing procedure cannot achieve gross resection of tumor while still preserving a useful extremity, because amputation improves only local control and does not address distant disease.

Further improvement in survival in this group of patients will be dependent on better systemic treatment for extremity soft tissue sarcoma, and not on more radical surgery.

 Williard WC, Hajdu SI, Casper ES, Brennan MF.

                Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.

                Association of local recurrence with subsequent survival in extremity soft tissue sarcoma

The aim of this study was to analyze local recurrence and its correlation with subsequent metastasis and disease-specific survival in extremity soft tissue sarcoma

                Journal of Clinical Oncology, by American Society of Clinical Oncology

                JJ Lewis, D Leung, M Heslin, JM Woodruff and MF Brennan
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

                Association of local recurrence with subsequent survival in extremity soft tissue sarcoma

findings suggest that there is a strong association of local recurrence with the development of subsequent metastasis and tumor mortality, and that local recurrence is a poor prognostic factor.

patients who develop local recurrence and have high-grade tumors as being at high risk for systemic disease.

                Journal of Clinical Oncology, by American Society of Clinical Oncology

                JJ Lewis, D Leung, M Heslin, JM Woodruff and MF Brennan
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

Hyperthermic Isolated Limb Perfusion for Extremity Sarcomas

Results: All patients had initial complete clinical responses to HILP, and the limb was salvaged in 4 of the 5 patients. Complications included chronic lymphedema, neuropathic pain, and prolonged wound healing.

Conclusions: HILP with melphalan is a safe and effective treatment option for selected patients with locally advanced and unresectable extremity sarcomas. The response rates are high, with limb salvage occurring in most patients.

Cancer Control: Journal of the Moffitt Cancer Center

Christina J. Kim, MD, Chris Puleo, PA-C, G. Douglas Letson, MD,

Complications and outcome of external hemipelvectomy in the management of pelvic tumors

     

Results: Postoperative complications occurred in 36 (53%) patients

flap necrosis in 11 (16%),

wound infection in 24 (35%),

other complications in 12 (18%).

Four (6%) patients died postoperatively.

average hospital stay after curative versus palliative resection was 39 versus 24 days.

Local recurrence is 35% of the patients.

The estimated 5-year survival for curatively resected patients was 21%.

Annals of Surgical Oncology

                Justus P. Apffelstaedt^{2, 3}, Deborah L. Driscoll², James E. Spellman², Augustine F. Velez², John F. Gibbs² and Constantine P. Karakousis¹

<!--[if !supportLists]-->n  <!--[endif]-->Complications and outcome of external hemipelvectomy in the management of pelvic tumors

     

                Conclusions: External hemipelvectomy is a procedure with considerable morbidity and is indicated for only a minority of far-advanced tumors. It offers a chance of palliation and possibly cure when lesser surgical options have been exhausted

Annals of Surgical Oncology

                Justus P. Apffelstaedt^{2, 3}, Deborah L. Driscoll², James E. Spellman², Augustine F. Velez², John F. Gibbs² and Constantine P. Karakousis¹

THANK YOU!

REFERENCES

SCHWARTZ'S PRINCIPLES OF SURGERY - 8th Ed. (2005)

The National Cancer Data Base report on soft tissue sarcoma. Cancer 78:2247, 1996. 3. Lawrence W Jr., Donegan WL, Natarajan N, et al:

Adult soft tissue sarcomas. A pattern of care survey of the

American College of Surgeons. Ann Surg205:349, 1987. 4. Coindre JM, Terrier P, Guillou L, et al:

MCQ

1. What is the most important prognostic factor for patients with sarcomas.

2. According to AJCC Staging System, Nodal disease is designated as

1. stage I
2. stage II
3. stage III
4. stage IV

3. Soft tissue sarcomas most commonly metastasize to the

1. Bones
2. Lungs
3. Brains
4. liver

1. The current version of the American Joint Committee on Cancer staging criteria for soft tissue sarcomas relies on

1. histologic grade
2. tumor size
3. depth
4. distant or nodal metastases

2. The following subtypes has a higher rate of lymph node metastasis in soft tissue sarcomas

1. Rhabdomyosarcoma
2. epithelioid sarcoma
3. malignant fibrous histiocytoma
4. liposarcoma

3.  Oncogenes are genes that can induce malignant transformation and tend to drive cells toward proliferation. The following  oncogenes have been identified in association with soft tissue sarcomas.

1. c-erbB2
2. retinoblastoma (Rb)
3. Nmyc
4. P53