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PubMed1996

[Head and neck sarcoma].

We reviewed patients with primary soft tissue and bone sarcomas treated in our department. Malignant tumors arising in somatic, connective, soft tissues, especially those of the head and neck, are uncommon and few reports have been published. Between 1980 and 1995 15 patients with head and neck sarcoma were seen. The major sites involved included: paranasal sinuses and orbits (2 patients), neck (4) and 1 each in the forehead, temporal region, mandible, parapharyngeal space, tongue, hypopharynx, larynx, cervico-mediastinal region and nasal cavity. Follow-up ranged from 1-16 years. The youngest patient was a 12-year-old male. Rhabdomyosarcoma, one of the most common mesenchymal tumors found in the head and neck, especially in children, was excluded from our study because of its different clinical behavior and its chemosensitivity, unlike other sarcomas. Most of our patients were treated with wide-field resection; 12 received postoperative radiation, and 3 combined surgery and chemotherapy. Wide excision with negative margins is considered an important, positive, prognostic factor, as the extent and adequacy of excision largely determines survival and the incidence of local recurrence. All our patients underwent initial, wide, local excision of the lesion. Our observations strongly suggest that wide resection is the most effective means of therapy for sarcoma of the head and neck. Radical neck dissection was not only performed for manifest regional neck metastases, but was also used for completing the enlarged, wide-excisions of liposarcomas. The worst survival was in patients with osteosarcoma, angiosarcoma and fibrosarcoma, with an absolute 5-year survival of 60% (9/15).

Adult↗
PubMedDate not supplied

[Treatment of retroperitoneal sarcoma].

AIM: To evaluate the results of treatment of retroperitoneal sarcomas. PATIENTS AND METHODS: We evaluated clinical, diagnostic, surgical and histological parameters as well as adjuvant therapy and evolution in 15 patients with retroperitoneal sarcoma. RESULTS: All patients presented abdominal tumors at diagnosis. Imaging revealed retroperitoneal origin. Complete surgical resection was carried out in seven patients (47%), reduction in size of tumor mass in five (33%) and exploratory laparotomy in three (20%). Histological analysis revealed 12 liposarcoma (80%), two leiomyosarcoma (13%) and one fibrosarcoma. Adjuvant therapy was given to two patients. Of the patients who underwent complete surgical resection, four survived without relapse (1, 2, 5 and 5 years respectively). With the other treatments, there were no survivals at 2 years. Adjuvant therapy did not influence survival. CONCLUSIONS: Retroperitoneal sarcoma is diagnosed late. Prognosis is poor due to tumor relapse and only complete surgical removal produces a "cure".

Adult↗
PubMed1989

Primary malignant giant cell tumor of bone: "dedifferentiated" giant cell tumor.

Well documented examples of primary malignant giant cell tumor of bone (giant cell tumor and concurrent sarcoma arising de novo) are exceedingly rare in the literature. We report a case arising in the left ischium of a 44-yr-old man. He had no previous history of radiation therapy or multiple resections. Histologically, the tumor was a typical giant cell tumor of bone juxtaposed to a malignant fibrous histiocytoma (MFH). The juxtaposition of a high grade sarcoma (MFH) and a locally aggressive nonmalignant neoplasm such as giant cell tumor is analogous to several other tumors of bone and soft tissue in which a low grade malignant or locally aggressive tumor can be associated with MFH or fibrosarcoma de novo, namely chondrosarcoma, chordoma, liposarcoma, and well differentiated intraosseous and parosteal osteosarcoma. The presence of a high grade malignant component in each of the aforementioned neoplasms generally portends a more ominous prognosis, although this is not invariably true. Recognition of the phenomenon of "dedifferentiation" (or tumor progression) in some bone tumors and sarcomas is important to ensure appropriate treatment. Distinction from secondary malignant giant cell tumors which are usually radiation induced is also important, since the latter have a much worse prognosis than those with dedifferentiation occurring de novo.

Adult↗
PubMedDate not supplied

In vitro and in vivo expression of somatostatin receptors in intermediate and malignant soft tissue tumors.

BACKGROUND: Previous studies showed that the subtype 2 of somatostatin receptors (sstr2) is generally expressed by soft tissue sarcomas. MATERIALS AND METHODS: Using reverse transcription-polymerase chain reaction (RT-PCR), the present study evaluated the expression of sstr mRNA in 31 human intermediate and malignant soft tissue tumors. In 8 patients somatostatin receptor scintigraphy was performed to detect soft tissue tumor sites and visualize in vivo sstr expression. RESULTS: Somatostatin receptors were identified in 84% of the tumors analyzed by RT-PCR analysis. sstr1 and sstr2 were the receptors most frequently detected. Somatostatin receptor scintigraphy was able to identify primary and/or metastatic sites in 7 out of 8 patients, with an overall sensitivity per site of 87%. Concordant results were found between in vitro and in vivo techniques. CONCLUSION: The wide expression of sstrs in soft tissue tumors suggest a relevant role for these receptors in diagnosis and may have therapeutic applications.

Adult↗
PubMed2004

[Retroperitoneal sarcomas: our experience].

BACKGROUND: Retroperitoneal soft tissues sarcomas (STS) are relatively uncommon and constitute a difficult management problem. Although surgical resection is often difficult or impossible, current chemotherapy is not effective and radiation is limited by toxicity to adjacent structures. Thus, complete surgical resection remains the most effective modality for selected primary and recurrent disease. PATIENTS AND METHODS: Fifteen patients with retroperitoneal STS were admitted and treated between January 1990 and January 2003, and prospectively followed. Nine patients underwent complete surgical resection of 8 malignant and 1 benign tumor. Two patients underwent incomplete surgical resection of 1 malignant and 1 benign tumor. Patient, tumor, and treatment variables were analyzed for disease-specific and disease-free survival. RESULTS: The patients with unresectable disease, incomplete resection, and high-grade tumors presented significantly reduced survival time. In this study, stage at presentation, high histologic grade, unresectable primary tumor, and positive gross margin are strongly associated with the tumor mortality rate. CONCLUSIONS: Patients approached with curative intent should undergo aggressive attempts at complete surgical resection. Incomplete resection should be undertaken only for symptoms relief. Because death often occurs as a result of local progression in retroperitoneal liposarcomas, it is possible that incomplete resection may be beneficial in this histologic type. Complete surgical resection is the most effective modality for the treatment of retroperitoneal sarcomas.

Adult↗
PubMed1999

Primary retroperitoneal soft tissue sarcomas: results of aggressive surgical treatment.

A retrospective study was undertaken to evaluate the results of surgical treatment in a series of patients with primary retroperitoneal sarcomas consecutively treated by the same surgical team. The hospital records of 42 patients with primary retroperitoneal sarcomas who underwent surgical exploration at our unit from 1984 to 1995 were reviewed. A univariate analysis was used to identify the main clinical, pathologic, and treatment-related factors affecting long-term survival. Twenty-five patients (59.6%) underwent radical surgery. The 5-year survival and 5-year disease-free survival after radical resection were 48.1% and 38.8%, respectively. According to the univariate analysis of survival tumor classification (T), stage and gross surgical margins significantly affected prognosis. The study indicates that even though there are predetermined and unmodifiable tumor-related factors, such as tumor classification (T) and stage, that influence survival in patients with retroperitoneal sarcomas, wide surgical excision offers a concrete chance for long-term survival.

Adolescent↗
PubMed1998

Survival in patients with primary soft-tissue sarcomas treated within 6 years.

Since 1988, treatment strategies for our sarcoma patients have been determined by the same team and operations performed by one surgeon. The aim of this study was to analyse prognostic data on local recurrence and survival of 101 consecutive patients who presented in our institution with the primary tumour manifestation. After a median follow-up of 35 months, the local recurrence rate was 13.5%, the mean survival time was 68 months and the 5-year survival rate was 83%. Besides positive lymph nodes (only 3 patients) the quality of resection significantly influenced local recurrences (P < 0.05). Univariate predictors of mortality were tumour grade (P < 0.01), tumour size (P < 0.05), distant metastases (P < 0.01), and resection quality (P < 0.01). Multivariate predictors of mortality consisted of grade (P < 0.0001), positive lymph nodes (P < 0.001) and resection quality (P < 0.01). In this homogeneous group of patients, excellent recurrence and survival rates could be achieved. An optimized surgical treatment not only reduces the rate of local recurrences but also augments survival time.

Adolescent↗
PubMed1992

Surgery for disseminated abdominal sarcoma.

Seventy-two consecutive patients with disseminated soft tissue sarcoma in the abdomen were prospectively placed in a program of debulking surgery. The tumor was completely resectable in 64% of the patients. Following the first exploration, the median survival was 23 months for those with resection of metastases and 9 months for those without resection (p less than or equal to 0.01); the former group had a survival rate of 28% at 3 years, 18% at 4 years, and 4% at 5 years (44%, 37%, and 10%, respectively, for low-grade sarcomas, i.e., grade I or II sarcomas), whereas in the latter group, none survived for 3 years. In the group with resection, patients with grade III tumors had a median survival longer by 6 months, and those with low-grade tumors by 28 months (p less than or equal to 0.001), over the respective median survival of patients with unresectable tumors. Metastasectomy appeared to prolong survival in all patients and significantly so in patients with low-grade tumors and those with long disease-free intervals.

Abdominal Neoplasms↗
PubMed1989

The use of magnetic resonance imaging and spectroscopy in the assessment of patients with head and neck and other superficial human malignancies.

The proper demarcation of diseased tissue is important for radiation therapy planning and treatment. The volume to be irradiated is usually identified on radiographs or on x-ray computed tomography (CT) sections. Magnetic resonance (MR)-derived images of the proton T2 relaxation times in small pixel elements, typically 0.5 mm2 or less, provide significantly sharper differentiation between normal and diseased tissue. The T2 values in tissue depend on the tissue composition, histologic condition, and physiologic environment within the tumor. Furthermore, for many tumors the histogram of T2 values has a clear biphasic distribution suggesting that T2 maps may be useful for the identification of necrotic or hypoxic regions within tumors. The distribution of T2 values within the tumor bed shows the general pattern that the T2 values are elevated with a range greater than that seen in normal muscle. Elevated T2 values are not by themselves diagnostic of malignancy; however, they demonstrate the heterogeneity of the microenvironment present within a tumor. The spatial distribution of T2 values is being explored as a method for computer assistance in the delineation of the target volume for treatment planning. In addition, MR P-31 spectroscopic examinations were performed on 30 patients with squamous cell carcinomas of the head and neck. Although hampered by muscle contamination in some P-31 spectra obtained with surface coil profile localization techniques, significant trends can still be appreciated in our data. These trends include the following: (1) the P-31 spectra from malignant tissue have well-resolved spectral lines in the upfield region that correspond to Pi, phosphomonoester (PME), and phosphodiester (PDE) not usually seen in normal muscle; (2) the PDE/B-ATP and PME/B-ATP ratios are greater than unity in all cases; and (3) most of the tumors have higher PME peaks than PDE peaks. The P-31 spectra from patients treated with ionizing radiation changed during and after therapy. Some of the changes could be associated with alteration of the tumor metabolic activity or synthesis and breakdown of lipoproteins. These studies suggest that magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) studies may be useful for both radiotherapy treatment planning and the noninvasive monitoring of patients both before and during treatment.

Carcinoma, Squamous Cell↗
PubMed2000

[Malignant mesenchymoma of mesentery. A controversial entity].

We report a case of malignant mesenchymoma developed in the mesentery in a 62-year-old man with no past history. It was an huge mass in the mesentery of the ileum. The tumor was composed of areas of chondrosarcoma and rhabdomyosarcoma in addition to an undifferentiated fusiform component. Malignant mesenchymoma is a rare sarcoma occurring preferentially in the retroperitoneum or the thigh. The location in the mesentery is exceptional. Malignant mesenchymoma is discussed more particularly with dedifferentiated liposarcoma with heterologous elements. The patient is alive without recurrence three years after surgery.

Abdominal Neoplasms↗
PubMedDate not supplied

[Retroperitoneal liposarcoma].

Liposarcoma is the most frequent histotype of the rare and malignant retroperitoneal tumours. This neoplasm has a remarkable tendency to recurrence after surgical excision, rarely to metastasize. Recurrence usually shows a more aggressive behaviour than primitive disease with a higher tendency to penetrate into adjacent organs. The symptomatology often appears late and the first sign is frequently a palpable abdominal mass. Preoperative study involves using CT and MRI. The surgical resection is the only tool able to modify natural history with regard to survival and local recurrence. Prognosis is severe, with a survival of 5 years, variable from 12 to 50% in the different series. A very important prognostic factor is the degree of tumour and radical surgical treatment. From 1990 to 2001, 32 operations for malignant retroperitoneal tumors were performed by our surgical unit in 19 patients; in 7 of them the tumor was a liposarcoma (4 male and 3 female). Total operations for retroperitoneal liposarcoma were 15, in 4 patients a second operation was performed for recurrence, in 3 a third operation and in 1 a fourth. Eleven cases out of 15 have undergone exeresis of sarcoma. Average survival consisted in 4 years and 2 months. Intraoperative radiotherapy, performed in 3 patients, perhaps is able to offer some advantages in relation to local control of the disease and of the survival. A possible resection of eventual recurrence justifies a early follow up with CT and MRI.

Female↗