471. a clear cell sarcoma – a clinical case

S178 ABSTRACTS

Poster Session: Soft Tissue Sarcoma

468. A recurrence of abdominal liposarcoma e Clinical case

M. Martins1, A.C. Carvalho1, D. Brito1, T. Santos1, V. Castro1,

J. Oliveira1, H. Mesquita1, J. Monteiro1, J. Pinto Correia1

1 Centro Hospitalar do Alto Ave, General Surgery, Guimaraes, Portugal

Background: Liposarcoma (LS) is the most common histological type

of sarcoma. There are five subtypes (well differentiated, myxoid, dediffer-

entiated, pleomorphic, mixed type).

Material and methods: Description of the case of a patient with ane-

mia, pain and swelling in the left flank by LS abdominal conditioning.

Results: A 59 year-old Portuguese female with history of gastric

sleeve, 1 year ago, was admitted with throbbing pain in the right flank

associated with distention and swelling bulky for 1 month of evolution. Re-

ported nausea, asthenia and anorexia. Previous analysis revealed haemo-

globin of 7.2 mg/dl. After admission and red blood cell transfusion,

abdominal tomography does reveal a large mass (25 � 12cm) adjacent

to the anastomosis. The deterioration of the patient’s condition lead to

excision of abdominal mass adherent to the spleen (splenectomy block),

whose history revealed pleomorfic liposarcoma. Postoperative pancreatic

fistula has treated conservatively. Asymptomatic at the time discharge.

In oncology where it is decided not to radiotherapy or chemotherapy.

Conclusions: The LS grows slowly and it’s diagnosed only when it be-

comes larger. The recommended treatment is complete resection even if

necessary adjacent organs. Radiotherapy or chemotherapy is not effective

in increasing survival.

No conflict of interest.

http://dx.doi.org/10.1016/j.ejso.2014.08.456

469. Oncovascular surgery: A new perspective facing advanced

retroperitoneal and pelvic malignancies

R. Pau1, M. Merlo2, R. Piana3, S. Sandrucci4

1 University of Turin Surgical Oncology, Vascular Surgery, Turin, Italy2 Citt�a Della Salute E Della Scienza, Vascular Surgery, Turin, Italy3 Citt�a Della Salute E Della Scienza, Orthopedic Surgery, Turin, Italy4University of Turin Surgical Oncology, Turin, Italy

Background: Surgical resection remains the cornerstone for the cura-

tive treatment of oncological disease. When a tumour mass encases a crit-

ical arterial or venous structure, successful symptom relief and long-term

oncological control may be achieved through careful preoperative planning

within a multi-disciplinary team incorporating oncological and vascular

specialists. To highlight the strategic issues pertaining to the vascular man-

agement of these patients, this review addresses the principles in planning

oncovascular surgery, namely where cancer resection necessitates concur-

rent ligation or reconstruction of a major vascular structure.

Material and methods: We considered 8 patients, 4 with a sarcoma of

the vena cava, two with a recurrent cervical carcinoma and two with a

retroperitoneal pelvic sarcoma. In three cases a vascular prosthesis was

employed, in the other cases a direct ligation or a tangential resection

was performed.

Results: After a 24 months mean follow-up one patient locally

recurred. In two cases distant metastases were observed after 4 and 9

months after the intervention. In one case we had a venous kidney infarc-

tion after one month from surgery. In all cases a slightly improvement of

referred compressive symptoms was observed; QoL improvement was not

assessed.

Conclusions: Major vessel involvement of a tumour mass should not

necessarily be considered a barrier to en bloc resection and hence curative

surgery. Radical surgical resection may offer the only chance for cure or

palliation for these patients. Detailed preoperative planning within an

extended multi-disciplinary team that includes vascular specialists is

essential for these complex patients. The observed outcomes for different

malignancies suggest that survival is dependent upon complete clearance

of the primary pathology and tumour biology rather than vascular-related

complications.



470. Surgical treatment of retroperitoneal sarcomas. Ten years’

experience

A. Petrou1, K. Neofytou2, S. Corsava3, A. Papalambros4, M. Loizou1, E.

Felekouras4

1 Nicosia Teaching Hospital, Department of Surgical Oncology, Nicosia,

Cyprus2 The Royal Marsden NHS Trust, Department of Academic Surgery,

London, United Kingdom3 St George’s Medical School, London, United Kingdom4University of Athens Medical School, LAIKON Teaching Hospital, First

Department of Surgery, Athens, Greece

Background: Complete sarcoma resection, experienced aggressive

surgical technique and individualization of patient management is the

gold standard of treatment for patients suffering retroperitoneal

sarcomas.

Materials and methods: Clinical outcomes of primary retroperitoneal

sarcoma resections from January 2002 until January 2012, were reviewed

to determine the efficacy of complete surgical resection as the principle in-

strument for treatment without any radiotherapy or chemotherapy. The

duration of illness, histological type, tumor size and grade as well as organ

resection were recorded and subsequently reviewed. The surgeries took

place at the First Department of Surgery, University of Athens Medical

School, Athens, Greece, and at the Hepatobiliary and Surgical Oncology

Department, Nicosia Teaching Hospital, Nicosia, Cyprus.

Results: Our study included seventy-nine cases of sarcoma resections

(fifty-eight first-time laparotomies, sixteen second-time and five third-time

representing fifty-eight patients (33 male and 25 female). These resections

took place between 2002 and 2012. Most patients (95%) had complete

resection and 46 of them did not receive neither radiotherapy nor chemo-

therapy. 30 day mortality was zero. Patients who had had duration of

symptoms less than 3 months overall and their tumor size was less than

5cm and was histologically classed as liposarcoma low grade, had a five

year survival close to 100%. Patients with more than 3 months symptoms

duration, with high grade tumor, where tumor size was between 10e20 cm

or more and histological types were leiomyosarcoma, liposarcoma, malig-

nant fibrous histiocytoma or malignant peripheral nerve sheath tumor, had

an average 5-year survival of 35%.

Conclusions: R0/R1 surgical resection of retroperitoneal sarcomas

combined with individualized patient management when undertaken by

experienced surgical teams can succeed in treating patients without the

need of radiotherapy or chemotherapy adjuncts.



471. A clear cell sarcoma e A clinical case

M. Martins1, C. Longras1, D. Andr�e1, A.C. Carvalho1, T. Santos1, J.

Monteiro1, J. Magalh~aes1, A. Castro1, J. Pinto Correia1

1 Centro Hospitalar do Alto Ave, General Surgery, Guimaraes, Portugal

Background: The clear cell sarcoma is a rare type of primary cancer

that affects young adults between 20 and 40 years. Are more common in




ABSTRACTS S179

women and tend to grow along the tendons in the limbs, especially the

feet and hands. They are generally asymptomatic. A genetic cause is

cell sarcoma translocation gene. Clear cell sarcoma without translocation

may have other genetic mutations, currently unknown, causing the same

effect. Can be classified in two ways: by location or by type of translo-

cation. Types of clear cell sarcomas based on place of origin are: Clear

cell sarcoma typical of tendons and aponeurosis (Layers of tendons);

Clear cell sarcoma, gastrointestinal; Clear cell sarcoma of the skin

(Skin). By genetic classification, the most common types of transloca-

tions are EWSR1/ATF1 or EWSR1/CREB1. Some tumors have no

EWSR1 translocation.

Materials and methods: Describe the case of a 52 year old man pre-

sents about 3 nodular lesions on the dorsum with 3 months of evolution. It

is subjected to excisional biopsy that reveals clear cell sarcoma.

Results: A men, 52 years with hypertension is referred for outpatient

general surgery for 3 nodular lesions with about 5cm � 5 cm in the dorsal

region with 3 months of evolution. Held ultrasound lesion that was not spe-

cific. Chest computed tomography scan performed which showed two mi-

cronodules in the lung. Excisional biopsy of the lesion with confection of

skin flap and expanded margins was submitted. Histology revealed clear

cell sarcoma. In oncology consultation it was decided to start chemo-

therapy. The patient was asymptomatic at 3 months.

Conclusions: Treatment consists of surgery (wide local excision). As

clear cell sarcomas are usually invasive, surgery is the main treatment for

these lesions, aimed at removing the tumor with safety margins. Radia-

tion therapy is commonly used in the surgical area to kill microscopic re-

sidual tumor cells and thus reduce the chances of local recurrence. When

applied before surgery, radiotherapy can reduce the size of the tumor and

make minor surgical procedure. The most promising experimental strat-

egy for the treatment of clear cell sarcoma is a targeted therapy that is

designed to achieve specific characteristics of cancer cells. One type of

targeted therapy is the tyrosine kinase inhibitor that blocks hyperactive

signaling molecules in cancer cells that promote the growth of cancer

receiver.



472. Deep-seated huge hibernoma of soft tissue: A rare differential

diagnosis of atypical lipomatous tumour/well differentiated

liposarcoma

N. Vassos1, M. Lell2, W. Hohenberger1, R. Croner1, A. Agaimy3

1 University Hospital Erlangen, Department of Surgery, Erlangen,

Germany2University Hospital Erlangen, Institute of Radiology, Erlangen, Germany3University Hospital Erlangen, Institute of Pathology, Erlangen, Germany

Background: Hibernoma is a rare benign fat-forming soft tissue tumor

that differentiates similar to brown fat, hence an origin from remnants of

fetal brown adipose tissue has been proposed. Mainly young adults are

affected, usually without significant clinical symptoms.

Material and methods: We report on four patients with hibernomas,

who were treated at our hospital during the last 10 years. The clinicopath-

ologic and immunohistochemical features are presented and treatment and

follow-up data discussed.

Results: Patients were 2 women and 2 men aged 21e67 years (mean:

45 yrs) who presented with a slowly growing, painless mass. The anatomic

location was the thigh, upper arm, lateral thoracic wall and paravertebral

soft tissue. Two of them were diagnosed preoperatively through a percuta-

neous core needle biopsy and the other two underwent surgery because of

high clinical and radiological suspicion of liposarcoma. The tumor’s size

ranged from 7 cm to 15.5 cm (mean: 11 cm). All were deep-seated subfas-

cial intramuscular masses. Histologically, all four tumors were of the

typical variant. All patients underwent a R0-surgical resection of the tumor

and they were recurrence-free at last follow-up (mean:47 months; range:

25e87).

Conclusions: Hibernoma may present as huge deep intramuscular soft

tissue mass in adults, closely mimicking well differentiated liposarcoma

and should be considered in the differential diagnosis of fatty soft tissue

tumors in any location. Surgical excision is the treatment of choice. The

tumor has no malignant or recurrence potential.



Poster Session: Thyroid Cancer

473. Prognostic factors for disease-specific survival in 109 patients

with H€urthle cell thyroid carcinoma: A single-institution experience

I. Edhemovic1, R. Petric1, B. Gazic2, N. Besic1

1 Insititute of Oncology, Surgical Oncology, Ljubljana, Slovenia2 Insititute of Oncology, Pathology, Ljubljana, Slovenia

Introduction: H€urthle cell thyroid carcinoma (HCTC) is a rare disease.

It is believed that it is more aggressive than follicular thyroid carcinoma.

The aim of our study was to identify factors associated with disease-spe-

cific survival.

Patients and methods: Altogether 109 patients (27 male, 82 female,

median age 62 years, range 19e87 years) with HCTC treated at our insti-

tute from 1972 e 2011 were included in the present retrospective study.

Data on age, clinical and histopathology factors, tumor stage, recurrence,

disease-free and disease-specific survival were collected. Univariate anal-

ysis was used to identify factors associated with disease-specific survival.

Cox’s multivariate regression model was used to identify independent

prognostic factors for disease-specific survival.

Results: Of 109 patients, 12 (11%) had distant and 8 (7%) locoregional

metastases before the first treatment. A total or near-total thyroidectomy,

radioiodine ablation of thyroid remnant and external beam radiotherapy

was performed in 71%, 81% and 26% of patients, respectively. Recurrence

was diagnosed in 27 cases: locoregional, distant and both locoregional and

distant in 12, 12 and 3 cases, respectively. The follow-up period was 1 to

337 (median 107) months. By the end of the study, 68 patients were still

alive (52 had no evidence of disease, 16 were alive with disease), five pa-

tients were lost to follow-up, 16 patients died of causes unrelated to pri-

mary disease, while 20 patients died of thyroid carcinoma (18 of distant

metastases, 2 of distant and locoregional disease). The 5-year, 10-year

and 20-year disease specific survival were 95%, 88% and 62%, respec-

tively. Independent prognostic factors for disease-specific survival were:

age of patients, distant metastases and residual tumor after surgery. In pa-

tients older than 45 years of age the risk of shorter disease specific survival

was 6.5 times higher than in younger patients. In the patients with distant

metastases the risk of shorter survival was 3.3 times higher than in the pa-

tients without dissemination. The risk of shorter disease specific survival

was 21.9 and 5.1 times higher in patients with macroscopic and micro-

scopic residual tumor than in patients without residual disease,

respectively.

Conclusions: Long disease-specific survival was found in patients with

HCTC younger than 45 years of age, without distant metastases and

without residual tumor after surgery.






471. a clear cell sarcoma – a clinical case

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