Introduction

Angiosarcomas are malignant vascular tumors (₁). Different cutaneous vasculary proliferations such as benign lymphangiomatous papules, atypical vascular lesions, benign lymphangioendothelioma and containing malign lesions as high-grade angiosarcomas were reported at irradiated skin regions (₂). They frequently occured in subcutaneous tissues of skin and breast and deep soft tissues (₃). Angiosarcoma is one of the rarest form of soft tissue neoplasm and it was shown in the 20 year study held at M.D. Anderson Hospital that they have an insignificant rate in total vascular tumors and they comprise less than 1% of all sarcomas (₄).

Desmoid tumor is a benign lesion because its rarely shows extension. Management must be planned as low grade fibrosarcoma due to its local agressive behavior (₅). Desmoid tumors mostly invades the abdominal wall. Although chest wall invasion is rare, it can invade nerve and vascular tissues. Adjacent organ invasion can cause mortality. Despite these, 10 years survival is over 90% (₆,₇).

Case 1

The patient was a 30 year old man and admitted to our clinic for a big, enlarging and aching mass at right upper quadrant of the chest anterior wall, for the last 3 months. We determined that he was treated with combined chemotherapy and radiotherapy for a year, 13 years before, for Hodgkin lymphoma diagnosis. Relevant oncology specialist reported that complete remission was achieved, in the annual visits. All system findings were normal in physical examination and there wasn't any additional pathologic finding in biochemical parameters. We inspected and palped a nonpulstatil, solid, 10x15 cm mass on right upper quadrant of chest anterior wall.

Diagnostic imaging investigations were withheld respectively and in chest X-ray we observed a homogenous, 10x7 cm mass between the 2 ^nd and 5 ^th rib, extending convexly to right lung, adjacent to the mediastinum of the right hemithorax upper zone (Figure 1).

Following this investigation we performed a soft-tissue ultrasonography directed to relevant localization and found a 10x12 cm mass, arising from right superior-anterior mediastinum and extending subcutaneously, having a heterogenous solid inner structure with cystic and calcific areas and an appearance on sternum probably due to destruction and prodiagnosed as thymoma (?) or malign teratoma (?) (Figure 2).

Figure 1

Figure 1: Chest X-ray of our first case.

Figure 2

Figure 2: Image of soft tissue ultrasonography of our first case.

Figure 3

Figure 3:Thoracal MR images of our first case.

Figure 4

Figure 4: Imagination of the infiltration in right pectoralis majus muscle with thoracal MR.

Figure 5

Figure 5: Image of the destruction of the manubrium sternii.

The next method was contrasted thoracic MRI and we determined a space-occupying lesion localized at the right clavicle distal end's cauda and thorax anterior wall (a suspicious infiltration at clavicle's distal end) (Figure 3).

Figure 6

Figure 6: This image showed a close neigbouring with right brachiocephalic artery and vein and brachiocephalic truncus.

Figure 7

Figure 7: Right 1 and 2 ribs were in the mass.

Figure 8

Figure 8:Macroscopic exploration of the mass after decolation.

Figure 9

Figure 9: Open defect was repaired secondarily with 4x6 cm goretex patch.

Figure 10

Figure 10: Reconstruction with left pectoral myocutoneous flap.

The lesion was at the medial line and at the right of it. Its largest axial dimension was 10x8 cm. Craniocaudal extension was approximately 8cm. It was showing heterogenic signal changes in all sequences and a heterogenic type strong uptake pattern (Figure 3). It was partially invaded the clavicle's distal part from right caudal region. It deplaced the pectoral muscle to anterior in the right (infiltration in right pectoralis majus muscle, Figure 4). It was seen to destruct the manubrium sternii and deplaced it to the left side of medial line (Figure 5). Also it was showed a close neigbouring with right brachiocephalic artery and vein and brachiocephalic truncus. It was extending to distal part of ascending aorta at caudally in anterior mediastinum and very adjacent to vascular structures (Figures 5 and 6). Lesion was mostly originating from manubrium level and extending exophytically to the right side of medial line (Figure 4 and 5). Both axillary regions were seen clearly. Right 1 ^st rib was in the mass and had an cranial indentation at 2 ^nd rib (Figure 7). Last imaging investigation was aortography and it showed that there wasn't contrast diffusion in right upper hemithoracal mass. Additionally we performed brain CT, whole abdominal USG and whole body bone scintigraphy and couldn't find a primary neoplastic or metastatic focus. Patient was undergone operation for extirpation of giant, solid, heterogeneic mass at right anterior-superior hemithorax.

During operation, skin and muscle tissues surrounding the mass was decolated and pseudocapsular structures were peeled – off (Figure 8). The mass invaded the 1 ^st and 2 ^nd ribs, ½ medial of clavicle, 1/3 lateral part of manubrium sterni and sternoclavicular joint and the destruction was significant in these structures. We completed the radical excision of the mass. Also destructed anterior segments of 1 ^st and 2 ^nd ribs and anterior ½ medial part of the clavicle were removed with excision. Open defect due to removal of pleura adjacent to mass was repaired secondarily with 4x6 cm goretex patch (Figure 9). Skin defect at right infraclavicular region reconstructed with left pectoral myocutoneous flap closure (Figure 10). Patient was discharged on the 8 ^th postoperative day with surgical cure. In the interpretation of hystopathologic investigation it was determined to be an postirradiation angiosarcoma due to Hodgkin disease therapy and that bone tissues were infiltrated (moderately differantiated, grade II). After the first postoperative mounth, patient was followed and treated at Haematology and Radiation Oncology outpatient clinics.

Case 2

Our patient was a 36 year old man and he had right shoulder pain during the last 1.5 years and last 6 months he had swelling at the right anterior chest wall and neck and almost totally mobility limitation of the right arm (Figure 11). In another institution an incisional biopsy was performed for giant mass and with fibromatosis (extraabdominal desmoid tumor) prediagnosis he was referred to our clinic for further evaluation and treatment. Vital signs were normal, there was a firm, fixed and painless mass extending to the axillary fossa and right supraclavicular area from the right anterior chest wall pectoral muscle and an incision scar was seen on it (Figure 11). Other system examinations and biochemical parameter results were in normal range. Radiodiagnostic evaluations showed increased density of the inferior neck region and right lateral part of thorax superior outlet in the chest X-ray and it was determined that the lesion compressed the upper lobe of the right lung and caused a volume decrease of the right lung. Thorax axial computerized tomography (CT) sections showed that at the right lateral part of the thorax wall, posterior to pectoral muscle and anterior of scapula there was a localized, lobulized mass lesion (Figure 12). Dimensions of the mass were 141x70 mm at the widest part, it had a hypodense necrotic area at lateral region and also vascularity increased on its surface. There wasn't bone destruction and erosion. Therfore, it was thought that it is correlated with malign soft tissue tumor. Additionally we observed extrinsic compression of the right lung and volume decrease due to the mass. A lobulated contoured solid mass was seen with 20 cm cranio-caudal length, 9 cm. transverse and 14 cm anteroposterior width at the right supraclavicular fossa, axillary fossa and thorax lateral wall by contrasted 3D Inflow Cervical Thorax MR and MR angiography. The mass was homogenous and extended from posterior clavicula to thye inferior cervical region and reached the anterior scapula posteriorly (Figure 13). It was slightly expanded to the lung from intercostal regions but didn't invade pleura or costas. It was very close to vertebral colon at inferior neck and didn't extend to spinal cord. Brachial plexus was invaded (Figure 14). The mass concentrically surrounded the subclavian and axillary arteries and also the subclavian vein was occluded from the truncus. This investigation showed that there is a lobulated contoured solid mass at right lateral thoracal wall, axillary region and supraclavicular fossa, occlusion at subclavian and axillar veins due to compression and that the subclavian and axillar arteries are pushed. With these further investigations and incisional biopsy results, he underwent an operation with a giant soft tissue tumor prediagnosis at the right anterior chest wall.

Under general anesthesia a long oblique incision was performed on the giant mass from the medial clavicular region to anterior axillary line. The whole mass was explorated by decolating the cutaneous and subcutaneous tissues on the mass. One third medial part of the right clavicle was removed because it was attacked by the mass. Mass was divided into two main lobules at that region. Resection of mass from thorax anterior wall was continued. External compression to ribs was determined. We had to transsect the brachial artery to continue the mass resection. It was confirmed that brachial plexus was destructed in the mass and limitation of mobility at the arm was due to this destruction by peroperative neurosurgery consultation. After removing the mass with its capsule, the brachial artery was repaired with a saphenus vein graft interpositioning (Figure 15). After regional hemostasis two hemovac drains were placed and Plastic and Reconstructive surgeons reconstructed the defect by a pediculled myocutaneous flap from left pectoral major muscle (Figure 16). He stayed in the intensive care unit for one day and then transferred to the clinic. Histopathologic examination confirmed the fibromatosis (extraabdominal desmoid tumor). The Common Oncology Counseil decided to follow the patient at the Radiation Oncology Outpatient Clinic and Physical – Therapy and Rehabilitation Clinic taking into account that these lesions are rapidly growing, agressive and invasive but not metastizing benign lesions. He was discharged at the 8 ^th day by surgical cure.

Figure 11

Figure 11: Appearence of the patient with giant desmoid tumor at right anterior chest wall during first admittance.

Figure 12

Figure 12: Thoracal CT image of the mass at right anterior chest wall defined as giant soft tissue tumor.

Figure 13

Figure 13: Cervical-thoracal MR and MR angiography image of giant desmoid mass of our patient.

Figure 14

Figure 14: Brachial plexus invasion finding in MR and MR angiography (SCA:Subclavian artery, SCV:Subclavian vein).

Figure 15

Figure 15: Macroscopic appearence of giant mass approx. 8 kilograms, after total extirpation.

Figure 16

Figure 16: After reconstruction of anterior chest wall defect with pediculed myocutaneous flap from left pectoral major muscle, appearence of the defect.

Discussion

Angiosarcomas are malignant tumors that tumors that recapitulate many of the functional and morphologic features of normal endothelium (₁,₄,₈). While highly differentiated forms resemble hemangiomas, it is difficult to distinguish the anaplastic types from carcinoma or melanoma. The term angiosarcoma is used for all sarcomas showing endothelial differentiation regardless of whether the lesion is related with vascular or lymphatic endothelium (₉).

In the past it was shown that intraabdominal or abdominal wall angiosarcomas have occurred following radiotherapy for cervix, ovary or uterus carcinomas. Same event occurred at different localisations after radiotherapy for different malign or benign conditions (₄,₈). Etiologic factor in our patient was radiotherapy performed to neck and upper thoracal region for Hodgkin disease., 13 years before.

Desmoid tumor is classified as malign bone tumor but because its metastatic spread is very rare its evaluated as benign lesion. They are treated as low grade fibrosarcoma due to local agression (₅,₆,₁₀).

Primary tumors are the most prevalent pathologies needing chest – wall resection. They are originated from soft tissue, bone and Clinical Symptoms are ranged between asymptomatic picture, pain, ulcus and infection (₁₀). Therapy must be planned by cardiovascular surgeon, plastic surgeon and neurosurgeons. We treated our patients with common consultation of this three disciplines.

Sarcoma occurrence at radiotherapy region for breast carcinoma or various malign neck tumors is rare. However they can be arise at bone or soft tissues. This type of tumors are announced so infrequently. Interval between radiotherapy and occurence of sarcoma is very variable and its generally over 10 years but rarely can be seen in 5 years (₁₁). In another study, the latent period between radiotherapy and diagnosis of vascular lesion was determined between 3 and 20 years (₂). Growing pattern of an epithelioid angiosarcoma is very similar to the carcinoma showing radiation changes (₉,₁₁).

The most widespread form is “angiosarcoma unassociated with lymphedema” meaning cutaneous angiosarcoma without lymphedema, in Weiss and Goldblum's (₁₂) classification and our case can be placed in this classification. As in our case it effects middle-aged patients and localising at head and neck region, containing postirradiation sarcomas.

When we take into account the last survival results of French Comprehensive Cancer Center, 9 of 20.000 conservatively treated breast carcinoma patients, had angiosarcoma (₁₃). It was determined that those lesions arised averagely 6 years after the ending of breast carcinoma therapy. Most of the patients didn't have lymphedema like our case, which shows a significant association with radiotherapy. Beginning of the lesions were understood with one or more swollen lesions and thickening of the skin or echimosis. Our case had initial findings as swelling, thickening and darkening lesions, too.

Key of successful therapy in chest wall tumors is early diagnosis and agressive surgical resection (₁₁,₁₃,₁₄). If major resection is planned for chest wall mass, they must be evaluated in detail, with history physical examination and further investigations and it must be determined if the lesion is primary or metastatic (₆,₇). Localization of the probable defect after resection, its depth, width, convenient tissue flaps and tissue amount necessary for reconstruction must be evaluated preoperatively (₁₅). Peroperatively defect reconstruction planning was made with Plastic and Reconstructive Surgery consultation.

Diagnostic approach in a patient with a probable chest wall tumor must contain; carefull history taking, physical examination, laboratory assays and following conventional chest radiography, soft tissue USG, computerized chest tomography (₁,₁₃,₁₄). Also magnetic resonance imaging (MRI) is a preferential method for primary chest-wall tumor. MRI not only distinguishes tumor, from nerve and blood vessels but also helps imaging from different planes as coronal and sagital, so we preferred this method too. Superiority of CT, can be seen during evaluation of lung parancim's metastatic diseases. Angiographical aortography as a further evaluation method gives us information about hemodynamy of the vascular lesion and has the best and most diagnostic value to describe the lesion's anatomical and functional work (₃,₁₆).

Chest wall and sternal tumors are for histopathological diagnosis during preop period. Because long-term prognosis determines the lesions etiology (₆,₁₇). Correctness of biopsy techniques are proportional with invasion level. Incisional biopsy during preoperative period is concluded as first choice with its excellent true diagnosis rate (₁₇).

The most important factor for successful therapy is a wide resection not preventing reconstruction in primary chest wall tumors (₆,₁₈). Surgical resection with 2 cm limitation in chest wall's benign tumors and low grade tumors are accepted as convenient procedures. Radiotherapy and / or chemotherapy can be used as adjuvant therapy.

Primary repair or reconstruction of defect due to resection of chest wall tumors, depends on localization, width and depth of the region (₈,₁₁). Reconstruction with plastic surgeon's help, at radical whole flat resection is really on marginal proedure. Soft tissue reconstruction of chest wall defects, frequently made with myocutaneous flaps and pectoralis major muscle is the most frequent flap for anterior and lateral chest wall defects (₁₅,₁₈). We used the same method in our patients for chest wall reconstruction. The most prevalent late complication of these tumors, needing reresection and reconstruction is cancer recurrence (₆,₇,₁₉). For prevention of recurrence or in the recurrence patients radiotherapy can be suggested after evaluating the general health, primary tumor and physiology of the patient (₂₀). Radiotherapy also is recommended for preventing potantial mortality in tumors, adjacent to neck or upper thorax airway. Comparing the morbidity of therapy and disease is necessary for radiotherapy (₂₀). Postoperative radiotherapy program was regular in our patients and there wasn't any recurrence at 4 ^th and 6 ^th months.

In the recent years there are developments in diagnosis and therapy of angiosarcoma and the most important one is vascular endothelial growth factor (VEGF) as announced by Fujimoto and assoc (₂₁). VEGF is an angiogenic cytokine and it is used in tumor angiogenesis study during recent years. While stimulating growth, VEGF also increases the permeability of endothelial cells. Some angiosarcomas can produce both VEGF and its receptors. This investigation showed that angiosarcoma can grow with both autocrine and paracrine ways. Although we can't monitorize the angiosarcoma therapy with serial VEGF serum concentration measures practically, hope full studies are going on. Additionally primary desmoid tumors of chest wall are very rare. Operation must be planned due to patient's lifestyle, working capacity and expecting prognosis and because reconstruction techniques are developed functional and cosmetic results are very good after radical resection. The most frequent late complication is cancer recurrence, so we recommend radiotherapy which is planned by Oncology Common Counseille.

Correspondence to

Doç. Dr. Ufuk YETKIN 1379 Sok. No: 9, Burç Apt. D:13 35220, Alsancak / IZMIR/TURKEY Tel: +90 505 3124906 Fax: +90 232 2434848 e-mail: ufuk_yetkin@yahoo.fr