Soft Tissue TumorsSoft Tissue Tumors

Sybile Val, MDDepartment of Surgery

SUNY Downstate Medical CenterSUNY Downstate Medical CenterOctober 29, 2009

Case PresentationCase Presentation

• 52 YOM presented to ED on 7/22 with52 YOM presented to ED on 7/22 with abdominal pain

Case PresentationCase Presentation

• PMH: • SocHx:PMH:– Schizophrenia

• PSH:

SocHx:– ½ PPD x 20 yrs

– Jehovah’s Witness

– None

• Meds:– Risperdal

• ALL:– NKDA

Case PresentationCase Presentation

• Vital:Vital:– 99.1 106/74 97 18 98%

• Exam• Exam:– Diffusely tender

• Labs:

103 1010

11

34284

136

4.9

103

26117

10

1.1

Hospital CourseHospital Course• HD #1 • HD#4HD #1

– GI consult

– Surgery consult

HD#4– Tachycardic 110‐120

• HD#5• HD#2

– EGD

– Transferred to surgical service

– Colonoscopy

– VIR biopsy

– Spiked to 101.5

– Cultures and CBC

17• HD#3– Started on diet

• Hct: 17

Path: Malignant small round blue cell tumor with extensive necrosis andtumor with extensive necrosis and degenerative changes

Hospital CourseHospital Course

• HD#6HD#6– Started on Epogen

• HD#10– Discharged home with surgical follow up

S i l Cli iSurgical Clinic

Pt asymptomatic

dHct increased to 30

Scheduled for resection

Hospital Course

• 8/23/09

Hospital Course

8/23/09– Pt admitted for bowel prep

• 8/24/09• 8/24/09– Exploratory laporatomy

E i f h i• Evacuation of hemoperitoneum

• Resection of small bowel mesenteric mass

• Small bowel resection x 2• Small bowel resection x 2

• Partial omentectomy

• Cholecystectomyy y

Intra-OperativelyIntra Operatively

Intra-OperativelyIntra Operatively

Intra-OperativelyIntra Operatively

Post-OperativelyPost Operatively

• POD#0 • POD#4– Hypotensive in RR– Central line placed

– Started on clears– Iron and Epogen

d– Started on pressors

• POD#1Weaned off all pressors

resumed

• POD#10– Hct 23– Weaned off all pressors

– Extubated in afternoon

• POD#3

Hct 23

• POD#11– Discharged home

– NGT removed– Hct 19

PathologyPathology

• Abdominal MassAbdominal Mass– 40x20x8cm in aggregate (19x11x5)

High grade spindle cell sarcoma– High grade spindle cell sarcoma

While Val @ VAWhile Val @ VA

• POD #49POD #49– Presented to ER with 3 days of abdominal pain associated with nausea and vomitingassociated with nausea and vomiting

– AVSS

– Exam: LLQ pain– Exam: LLQ pain

– Labs:93

1512

39402

130

5.4

93

30130.6

158

Hospital CourseHospital Course

• Admitted to surgical serviceAdmitted to surgical service– Conservative management x 2 days

Taken to the OR 8/15/09– Taken to the OR 8/15/09• Exploratory laporatomy

– carcinomatosis encountered

• Evacuation of hemoperitoneum (500ml)

• Lysis of adhesions

Hospital CourseHospital Course

• POD#6POD#6 – Extubated

• POD#7• POD#7– Started on clears

/– Made DNR/DNI by family

• Currently– Tolerating regular diet

– Awaiting hospice

Questions??

Soft Tissue Tumors

Soft Tissue SarcomaSoft Tissue Sarcoma

• Collective term for diverse group of malignanciesg p g• Arise from mesoderm• Represent 1% of all adult tumors• Represent 15% of pediatric tumors• Occur anywhere in the body

– 43%  extremities– 15%  retroperitoneum– 13% viscera13%  viscera– 10%  trunk

• More than 50 distinct histological subtypes

Soft Tissue SarcomaSoft Tissue Sarcoma

• Etiology is uncleart o ogy s u c ea• Tumor grade is the best indicator of

– Biological aggressivenessg gg– Metastatic potential– Grade is defined by:

• Tumor cellularity• Nuclear atypia• Degree of necrosisDegree of necrosis• Mitotic activity

• Present as asymptpmatic mass

• Indications for biopsy:Indications for biopsy:– Mass larger than 5cm

History of growth– History of growth

– Persists for more than 4 weeks

SURGERY is the FOUNDATION of the treatment of STS

For intra-abdominal or retroperitoneal sarcomas:

FNA or CNB  is not indicated

Except:Suspicion for lymphoma or germ cell tumor

Tissue diagnosis for preoperative therapy

Tissue dx for unresectable disease

S t d t t i f th iSuspected metastasis from another primary

• Poor prognostic factors:Poor prognostic factors:– Large size

Deep seated– Deep seated 

– Retroperitoneal vs. extremity

Hi h d– High grade

– High stage

P i i i– Positive margins

Specific Therapy by SiteSpecific Therapy by Site

• Extremity (43%)y ( )– Surgery alone

– Amputation

Limb Sparing Surgery and– Limb Sparing Surgery and adjuvant radiation

• Standard of care  • Retroperitoneal and intra‐abdominal (15%)abdominal (15%)– Surgery with negative margins

– Chemotherapy

– Radiation (??)• Adjuvant

• Preoperative

• Intra‐abdominal

• Regional LymphadenectomyRegional Lymphadenectomy– Not indicated

Only 2 6% of sarcomas metastasize to lymph– Only 2.6% of sarcomas metastasize to lymph nodes

Treatment OptionsTreatment Options

• Radiation Therapy • ChemotherapyRadiation Therapy– Recommended for high grade resected STS

Chemotherapy– Controversial

– Favorable px factors for – Improves local recurrence

No impact on survival

response to chemo:• Young Age

• Good performance status– No impact on survival

– Optimal timing unclear

• Good performance status

• Absence of liver mets

• Liposarcoma or synovial hi t lhistology

Recurrence Rates:Extremity 8 20%– Extremity 8‐20%

– Retroperitoneum 38‐50%

Could we have done somethingCould we have done something different in this case??

• Retrospective Analysis1991 2005– 1991‐2005

– Included only patients with advanced disease• Primary tumor or local recurrence not amendable to complete surgical resectionsurgical resection

• Presence of metastatic disease

• Goal:– Determine the efficacy of palliative chemotherapy

• Responders: those who achieved a complete or partial response• Non‐responders: All others

ll l d f f h l d h/l• Overall survival: measured from start of chemo until death/last follow up

– Determine whether specific factors influence the outcome of chemotherapyof chemotherapy

Median time to progression was

3 months

23% of pts were li @ 2alive @ 2 years

189/488

(39%)

299/488

(61%)

• Concluded:– Palliative chemotherapy in advanced STS should be regarded as a standard treatment option in the 

t f th i tmanagement of these aggressive tumors

– Doxorubicin based combinations regimens may be associated with superior survival to that achieved with psingle agent

– Factors predictive of superior survival included• Younger age

• Absence of bone mets

• Synovial or liposarcoma histology

Surgical resection is the most effective potentially curative therapy for soft tissue sarcomas regardless of origintissue sarcomas regardless of origin

In SummaryIn Summary

• Soft tissue sarcomas arise from mesenchymalSoft tissue sarcomas arise from mesenchymal tissues

• Extremities and retroperitoneum are most• Extremities and retroperitoneum are most common sites

MRI i f d di i i i d li• MRI is preferred diagnostic imaging modality

• Biospy may be considered for extremity lesions

• Surgical resection is mainstay of treatmentg y

Thank You…