case management, presentation, discussion and sharing of information on extremity sarcomas by...

CASE MANAGEMENT, CASE MANAGEMENT, PRESENTATION, DISCUSSION PRESENTATION, DISCUSSION

AND SHARING OF AND SHARING OF INFORMATION ON INFORMATION ON

EXTREMITY SARCOMASEXTREMITY SARCOMAS

byMichael Angelo L. Suñaz, M.D.

Department of SurgeryOspital ng Maynila Medical Center

CASE MANAGEMENT, CASE MANAGEMENT, PRESENTATION, DISCUSSIONPRESENTATION, DISCUSSION

E.A., 63/ME.A., 63/MBINAN, LAGUNABINAN, LAGUNA

CHIEF COMPLAINT: NON-HEALING WOUND ON THE RIGHT GLUTEAL

HISTORY OF PRESENT ILLNESS:HISTORY OF PRESENT ILLNESS:

4 months PTA, the patient noted a pimple-like lesion on his right gluteal area. No other associated signs and symptoms were noted.

3 ½ months PTA, the mass was noted to have increased in size. Consultation of a private physician was done and he was prescribed with unrecalled medications which afforded no relief.

2 weeks PTA, the mass persisted and was now associated with occasional pain and undocumented fever.

Persistence of his condition prompted consultation and subsequent admission.

PAST MEDICAL Hx:

unremarkable

FAMILY Hx:

HPN - paternal side

PERSONAL/SOCIAL Hx:

- no history of smoking or alcoholic beverage intake

PHYSICAL EXAMINATION:PHYSICAL EXAMINATION:

G/S: conscious, coherent, not in cardiorespiratory distress

BP= 120/70 CR=83 RR= 19 T=38.20C

SHEENT: no jaundice; pink palpebral cojunctiva,anicteric sclera, No NAD, No CLAD, No TPC

C/L: SCE, no retractions, clear BS

CVS: adynamic precordium, NRRR, no murmur

Abdomen: flat; soft; no palpable masses

Extremities: 10 x 13 cm firm, slightly movable, ulcerating mass, tender only upon deep palpation towards the right gluteal area

SALIENT FEATURES:SALIENT FEATURES:63 y/o, M10x13 cm firm, slightly movable,

rapidly growing ulcerating mass tender only upon deep palpation towards the right gluteal area

Occasional pain on the affected area Fever (38.20C)

10x13 cm firm, nontender, slightly movable, rapidly growing ulcerating

mass on the right gluteal area

•Rapidly growing

•With associated fever

•Tenderness only upon deep palpation in the direction of the gluteal area

•Rapidly growing

Infectious Neoplastic

Clinical Diagnosis:Clinical Diagnosis:

Diagnosis Certainty Treatment

Neoplastic Disease

75% Surgical

Infectious Disease

25% Surgical/

Medical

BASES:BASES:63 y/o, M10x13 cm firm, slightly movable,

rapidly growing ulcerating mass tender only upon deep palpation towards the right gluteal area

Occasional pain on the affected areaFever (38.20C)

Do I need a para-clinical diagnostic Do I need a para-clinical diagnostic procedure?procedure?

Paraclinical Diagnostic ProceduresParaclinical Diagnostic Procedures

Benefit Risk Cost Availability

Biopsy

Can provide a histopathologic diagnosis to determine the primary treatment of the lesion.

Bleeding

Readily available

Accurately delineates muscle groups and distinguishes between bone, vascular structures, and tumor. Sagittal and coronal views allow 3D evaluation of anatomical compartments.1

none ++++Not readily available

CT SCAN

Provide detailed survey of the abdomen and pelvis and delineate adjacent organs and vascular structures.1

Radiation Exposure

+++Not readily available

CT Scan of the Pelvis (9/29/08)– A mixed density mass with areas of

necrosis is seen arising from the right gluteus maximus muscle infiltrating into the subcutaneous fat measuring about 14 x 12.25 x 9.26 (CC x W x AP). The mass displaces the anal opening to the left.

CT Scan of the Pelvis (9/29/08)– There are no enlarged lymph nodes.– No osteolytic nor blastic changes seen.

Osteophytes are noted along the iliac margins and vertebral endplates.

– The included bowel loops, prostate and urinary bladder are unremarkable.

CT Scan of the Pelvis (9/29/08)

IMPRESSION: – Right gluteal mass, consider

sarcoma.– Tissue correlation suggested.– Degenerative osseous changes,

pelvis.

•Rapidly growing

Sarcoma

CXR (9/3/08)

Both lungs are clear.

The aorta is sclerotic.

The heart is not enlarged.

Diaphragm and sulci are intact.

IMPRESSION: Atheromatous Aorta .

Liver Ultrasound (9/3/08)

The liver is not enlarged. The ducts are not dilated. The echo pattern is homogenous. No focal mass lesion is seen.

IMPRESSION: Negative study.

Histopathology result (8/15/08)

GrossThe specimen consists of several

dark brown irregular soft and friable tissues, 4.0 cm in agrregate. The entire specimen is taken for study

Microscopic Microsections disclose loose aggregates of

malignant round cells exhibiting marked hyperchromasia, anisoneuclosis and prominent nucleoli. These have marked eosinophilia and moderate polymorphism. Some tumor giant cells are seen. These are admixed with necrotic and inflammatory material.

MALIGNANT ROUND CELL TUMOR, fragments of, admixed with abscess material.

•Rapidly growing

Sarcoma

Malignant Round Cell Liposarcoma

Pretreatment Diagnosis:Pretreatment Diagnosis:

Diagnosis Certainty Treatment

Malignant Round Cell Liposarcoma

95% Surgical/ Neoadjuvant,

Adjuvant Therapy

Gluteal Abscess 5% Surgical/ Medical

TREATMENTTREATMENT

PRETREATMENT DIAGNOSIS:

MALIGNANT ROUND CELL LIPOSARCOMA, RIGHT GLUTEAL

TREATMENTTREATMENT

GOALS OF TREATMENT:– Curative extirpation of the tumor

TREATMENT OPTIONSTREATMENT OPTIONSTREATMENT BENEFIT RISK COST AVAIL

En bloc Surgical Resection

Removal of the gross tumor.

Primary treatment modality.2

Local recurrence if done with inadequate margins.

Bleeding.

May require contiguous organ resection.2

++ Available

Pre-operative Radiation Therapy

Allows early multidiscipli-

nary planning while the tumor is in place.1

Allows lower doses to be delivered to an undisturbed tissue bed that is better oxygenated.1

Difficulty with pathological assessment of margins and increased incidence of wound complications.1

++++ Not readily available

Size of the pre-operative radiation fields and the number of joints included in the field are significantly smaller which may result in an improved functional outcome.1

Post-operative Radiation Therapy

Lower wound complication rate.

Larger radiation field.

++++ Not readily avalable

Brachytherapy Less radiation scatter and much shorter duration of therapy.2

Indicated only in the setting of high-grade lesions.2

Rates of wound complications similar to those of postoperative external beam radiotherapy.2

Adjuvant systemic chemotherapy

Statistically significant improvements in local recurrence, distal recurrence,and disease-free survival rates ranging from 6%-10%. 4% improvement in overall survival.2

Potential toxicity.2

++++ Available

Neoadjuvant systemic chemotherapy

Ability to assess tumor responsiveness to the give chemo-therapeutic agents, early treatment of metastatic disease, and downstaging of primary tumor.2

Potential toxicity ++++ Available

TREATMENT OF CHOICETREATMENT OF CHOICE

WIDE RESECTION AND POST-OPERATIVE RADIATION THERAPY

PREOPERATIVE PREPARATIONPREOPERATIVE PREPARATION

Informed consentPsychosocial supportOptimize patient’s healthScreen for any condition that will

interfere with treatmentPrepare materials

OPERATIVE TECHNIQUEOPERATIVE TECHNIQUE

Patient supine under CLEA Asepsis/Antisepsis Sterile drapes placed Intraoperative findings noted: Mass noted

to have extended partially to the serosal layer of the rectum and outermost layer of the sphincter muscle. Gluteus maximus muscle mass and sciatic nerve intact.

OPERATIVE TECHNIQUEOPERATIVE TECHNIQUE

Wide excision with 1 cm margin; flap created.

HemostasisPlacement of drainCorrect sponge and instrument

countApposition of flap with silk 2-0Dry sterile dressing

OPERATION DONE:OPERATION DONE:

WIDE RESECTION OF RIGHT GLUTEAL MASS

POST OPERATIVE DIAGNOSISPOST OPERATIVE DIAGNOSIS

Malignant round cell tumor (liposarcoma), right gluteal area

*Final histopathology report still pending

SHARING OF INFORMATIONSHARING OF INFORMATION

SARCOMASSARCOMAS

Refer to tumors that show evidence of mesenchymal differentiation.

1% of adult malignancies 15% of pediatric malignancies

• Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105

Account for nearly 50% of adult sarcomas.

Most common types :– Liposarcoma– Malignant Fibrous Histiocytoma (MFH)

Liposarcomas:– Well-differntiated– Myxoid/ round-cell– Pleomorphic

Diagnosis– Comprehensive history and PE– Mass is the most common presenting

complaint– Frequently, a trivial traumatic event draws

attention to the area (although there is probably no causal relation between a history of trauma and the development of a sarcoma).

Diagnosis– Core needle biopsy

Typically performed as the first step Can diagnose the presence of a sarcoma

and grade it in 80% of the cases. For histologic type, it has an accuracy of

Staging– Primary Tumor (T)

Tx – primary tumor cannot be assessed T0 – no evidence of primary tumor T1 – tumor is < or equal to 5 cm in its

greatest dimension• T1a – tumor is above the superficial fascia• T1b – tumor invading or deep to the superficial

fascia

• Delamn KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125

Staging– Primary Tumor (T)

T1 – tumor is > 5 cm in its greatest dimension

• T2a – tumor is above the superficial fascia• T2b – tumor invading or deep to the

superficial fascia

Regional Lymph Nodes (N)– Nx – regional lymph nodes cannot be

assessed– N0 – no regional lymph node

metastasis– N1 – Regional lymph node metastasis

Distant Metastasis (M)– Mx – distant metastasis cannot be

assessed– M0 – no distant mmetastasis– M1 – distant metastasis

Histopahological Grade (G)– Gx – Grade cannot be assessed– G1 – well-differentiated– G2 – Moderately differentiated– G3 – poorly differentiated– G4 - undifferentiated

Stage Grouping– Stage 1

A – G1-2, T1a-1b, N0, M0 B – G1-2, T2a, N0,M0

Stage Grouping– Stage II

A – G1-2, T2b, N0, M0 B – G3-4, T1a-1b, N0,M0 C – G3-4, T2a, N0,M0

Stage Grouping– Stage III

G3-4, T2b, N0,M0

Stage Grouping– Stage IV

Any G, Any T, N1, M0 Any G, Any T, N0, M1

En bloc Surgical Resection

Removal of the gross tumor.

Primary treatment modality.2

Local recurrence if done with inadequate margins.

Bleeding.

May require contiguous organ resection.2

++ Available

Allows early multidiscipli-

nary planning while the tumor is in place.1

Allows lower doses to be delivered to an undisturbed tissue bed that is better oxygenated.1

Difficulty with pathological assessment of margins and increased incidence of wound complications.1

++++ Not readily available

Size of the pre-operative radiation fields and the number of joints included in the field are significantly smaller which may result in an improved functional outcome.1

Post-operative Radiation Therapy

Lower wound complication rate.

Larger radiation field.

Brachytherapy Less radiation scatter and much shorter duration of therapy.2

Indicated only in the setting of high-grade lesions.2

Rates of wound complications similar to those of postoperative external beam radiotherapy.2

Adjuvant systemic chemotherapy

Statistically significant improvements in local recurrence, distal recurrence,and disease-free survival rates ranging from 6%-10%. 4% improvement in overall survival.2

Potential toxicity.2

++++ Available

Neoadjuvant systemic chemotherapy

Ability to assess tumor responsiveness to the give chemo-therapeutic agents, early treatment of metastatic disease, and downstaging of primary tumor.2

Potential toxicity ++++ Available

MCQMCQ

1. Sarcomas comprise how much of adult malignancies?

c. 15%

d. 20%

MCQMCQ

1. Sarcomas comprise how much of adult malignancies?

c. 15%

d. 20%

MCQMCQ

2. Sarcomas comprise how much of pediatric malignancies?

c. 15%

d. 20%

MCQMCQ

2. Sarcomas comprise how much of pediatric malignancies?

c. 15%

d. 20%

MCQMCQ

3. Extremity sarcomas comprise how much of adult sarcomas?

a. 10%

b. 30%

c. 50%

d. 20%

MCQMCQ

3. Extremity sarcomas comprise how much of adult sarcomas?

a. 10%

b. 30%

c. 50%

d. 20%

MCRMCR

A – 1, 2, and 3 are correctB – 1 and 3 are correctC – 2 and 4 are correctD – only 4 is correct E – none are correct

MCRMCR

I. Which of the following represents stage I soft-tissue sarcoma?1. G1-2, T1a-T1b, N0,M02. G1-2, T2b, N0, M03. G1-2, T2a, N0,M04. Any G, Any T, N1, M0

MCRMCR

I. Which of the following represents stage I soft-tissue sarcoma?1. G1-2, T1a-T1b, N0,M02. G1-2, T2b, N0, M03. G1-2, T2a, N0,M04. Any G, Any T, N1, M0

MCRMCR

I. Which of the following represents stage III soft-tissue sarcoma?1. G1-2, T1a-T1b, N0,M02. G1-2, T2b, N0, M03. G3-4, T2a, N0,M04. G3-4, T2b, N0, M0

MCRMCR

I. Which of the following represents stage III soft-tissue sarcoma?1. G1-2, T1a-T1b, N0,M02. G1-2, T2b, N0, M03. G3-4, T2a, N0,M04. G3-4, T2b, N0, M0

THANK YOU!!!

REFERENCESREFERENCES

Delman KA, Cormier JN: Soft-tissue and bone sarcoma, in Feig BW, Berger DH, Fuhrman GM (ed): The M.D. Anderson Surgical Oncology Handbook 4th ed . Philadelphia, Lippincott Williams and Wilkins, 2006, pp 125

Singer S, Canter RJ: Soft-tissue sarcoma, in Cameron JL (ed): Current Surgical Therapy 9th Ed. Philadelphia, Mosby, 2008, pp 1101-1105

JOURNAL CRITICAL APPRAISALJOURNAL CRITICAL APPRAISAL

Spinal metastases from myxoid Spinal metastases from myxoid liposarcoma warrant screening with liposarcoma warrant screening with magnetic resonance imagingmagnetic resonance imaging

Joseph H. Schwab, MDJoseph H. Schwab, MD 1 1, Patrick J. Boland, MD, Patrick J. Boland, MD 1 1, Cristina Antonescu, , Cristina Antonescu, MDMD 2 2, Mark H. Bilsky, MD, Mark H. Bilsky, MD 3 3, John H. Healey, MD, John H. Healey, MD 1 * 1 *

11Department of Surgery, Orthopedic Service, Memorial Sloan-Department of Surgery, Orthopedic Service, Memorial Sloan-Kettering Cancer Center, New York, New YorkKettering Cancer Center, New York, New York22Department of Pathology, Memorial Sloan- Kettering Cancer Center, Department of Pathology, Memorial Sloan- Kettering Cancer Center, New York, New YorkNew York, New York33Department of Surgery, Orthopedic and Neurosurgery Services, Department of Surgery, Orthopedic and Neurosurgery Services, Memorial Sloan-Kettering Cancer Center and Medical College of Memorial Sloan-Kettering Cancer Center and Medical College of Cornell University, New York, New YorkCornell University, New York, New York

email: John H. Healey (email: John H. Healey (healeyjhealeyj@@mskccmskcc.org.org))**Correspondence to John H. Healey, Department of Surgery, Correspondence to John H. Healey, Department of Surgery, Orthopedic Service, Memorial Sloan-Kettering Cancer Center, 1275 Orthopedic Service, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Suite A342, New York, NY 10021York Avenue, Suite A342, New York, NY 10021

ABSTRACTABSTRACT

Background:– Myxoid liposarcoma (MLS) has an

unusual tendency for extrapulmonary metastasis, particularly to the spine and soft tissues. The objective of this study was to determine the prevalence of spinal metastasis, treatment outcomes, and optimal screening method for spinal metastasis in patients with MLS.

ABSTRACTABSTRACT

Methods:– Data from patients with had spinal metastases

were obtained from the authors' institutional soft tissue sarcoma database. The accuracy with which positron emission tomography (PET) scans and bone scans identified metastatic lesions was compared with the accuracy of magnetic resonance imaging (MRI). Clinical response to treatment was based on pain, neurologic scores, and survivorship analysis.

ABSTRACTABSTRACT

Results:– There were 33 patients who developed

spinal metastasis after a median 36 months of follow-up (range, from 7.5 months to 33 years). Known spinal metastases were detected by bone scans in 16% of patients and by PET scans in 14% of patients.

ABSTRACTABSTRACT

Results:– Patients who underwent surgery had high-

grade spinal cord compression more often than patients who did not undergo surgery (72% vs 19%, respectively; P = .002). Pain and neurologic function were improved or maintained in all patients who received radiation alone (n = 8 patients) and in all but 1 patient who underwent surgery (n = 18 patients). The median overall survival was 51.4 months from the time of primary diagnosis and 21.9 months from the time of first metastasis.

ABSTRACTABSTRACT Conclusions:

– Bone scans and PET scan lack sufficient sensitivity to detect spinal metastasis from MLS. Treatment of metastasis is palliative, but local treatment can yield long-term disease control in select patients. Screening with whole-spine MRI may lead to the earlier detection of spinal metastasis. Cancer 2007. © 2007 American Cancer Society.

Appraisal Guide: Appraisal Guide: THERAPY OR PREVENTIONTHERAPY OR PREVENTIONAre the results of the study valid?

Primary Guides:

Was the assignment of patients to treatments randomized?

– NO. Data from patients with had spinal metastases were obtained from the authors' institutional soft tissue sarcoma database.

Primary Guides:

Were all patients who entered the trial properly accounted for and attributed at its conclusion?

YES. All 33 MLS patients with spinal metastasis were accounted for.

Primary Guides:

Was followup complete?

Appraisal Guide: Appraisal Guide: THERAPY OR PREVENTIONTHERAPY OR PREVENTION

Are the results of the study valid?

Primary Guides:

Were patients analyzed in the groups to which they were randomized?

Secondary Guides:

Were patients, health workers, and study personnel "blind" to treatment?

Secondary Guides:

Were the groups similar at the start of the trial?

Secondary Guides:

Aside from the experimental intervention, were the groups treated equally?

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