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Computed tomography of retroperitoneal and mesenteric sarcomas: a pictorial essay.

Retroperitoneal and mesenteric sarcomas are rare tumours that often do not present until they are very large. Computed tomography (CT) is helpful for diagnosis and for planning therapy. This pictorial essay illustrates the spectrum of CT manifestations of these tumours that the authors have encountered and includes a review of the various aspects of clinical presentation and treatment.

Fibrosarcoma↗

Desmin: its value as a marker of muscle derived tumours using a commercial antibody.

This study examined the staining reactions of a commercially available anti desmin antibody in 192 soft tissue sarcomas, 30 carcinomas and 22 malignant melanomas. Only 63% of rhabdomyosarcomas and 50% of leiomyosarcomas showed a positive reaction. Three non muscle sarcomas also reacted with the antibody. No positivity was seen amongst the carcinomas or the melanomas. These results do not compare favourably with the documented results of non commercial desmin antibodies in the literature. The most likely cause of the discrepancy in the varying results is the differing source of the antibody.

Adolescent↗

Sarcoma metastatic to the central nervous system parenchyma: a review of the literature.

Sarcoma metastatic to cerebral parenchyma, although rare, occurs more frequently than generally recognized. With increased duration of survival due to multi-modal therapy, more CNS metastases are being found. A literature search occasioned by a patient with metastatic sarcoma has produced some interesting results.

Antineoplastic Combined Chemotherapy Protocols↗

Myxoid liposarcoma of the supraclavicular fossa.

Liposarcomas generally originate most often in the extremities or retroperitoneum, less frequently in the head and neck, and rarely in the thorax. We describe a particularly rare presentation of myxoid liposarcoma originating in the supraclavicular fossa. The mass was resected and has not recurred. We searched our pathology database for other soft-tissue tumors of the supraclavicular fossa and found no other case of sarcoma originating in this site. In addition, we performed a literature review of thoracic and neck liposarcomas to identify similar cases and discuss their clinical course.

Aged↗

Improved survival for soft tissue sarcoma of the extremities by regional hyperthermic perfusion, local excision and radiation therapy.

Soft tissue sarcomas continue to present problems with both local control of disease and death from distant metastases after accepted surgical therapy. Multimodality management has been established in the literature to be superior to traditional therapy of wide excision or amputation. The results of the treatment of 64 patients at Oregon Health Sciences University using regional hyperthermic perfusion with l-phenylalanine mustard and actinomycin D, wide local excision and external radiation therapy are reviewed. Histologic tumor types included: 19 liposarcomas, 14 malignant fibrous histiocytomas, eight fibrosarcomas, five leiomyosarcomas, four malignant schwannomas and 14 other sarcomas of the extremities. Minimum follow-up study has been 24 months and the mean follow-up time is more than five years. One-third of the patients presented with local recurrent disease after unsuccessful primary treatment. Over-all local recurrence for all patients treated by a combination of the aforementioned modalities was 3.4 per cent at two years and 11.1 per cent at five years. The over-all survival rate for all patients was 88 per cent at two years and 67 per cent at five years compared with a published over-all five year survival rate of 41 per cent. Patients with malignant fibrous histiocytomas had both two and five year local recurrence rates of 7.7 per cent and survival rates at two and five years of 79 and 63 per cent, respectively. This is compared with a previously published over-all five year survival rate with malignant fibrous histiocytomas of 46 per cent. The results with the treatment of liposarcomas were also superior to the published literature. Not only are the treatment results with regional hyperthermic perfusions excellent for both primary and locally recurrent sarcomas of the extremities, but limbs previously considered unsalvagable can be spared.

Adolescent↗

Cytogenetics of tumors of soft tissue and bone. Implication for pathology.

Pathologists should be aware of the existence of diagnostically useful chromosomal rearrangements in several soft tissue and bone tumors. They include rearrangement of 8q12 in lipoblastomas, ring chromosomes in atypical lipomas, ring and giant marker chromosomes in well differentiated liposarcomas, t(12;16)(q13;p11) in myxoid liposarcomas, rearrangement of 7p21-22 in low-grade endometrial stromal sarcomas, t(2;13)(q37;q14) in alveolar rhabdomyosarcomas, t(X;18)(p11.2;q11.2) in synovial sarcomas, t(12;22) (q13;q13) in clear cell sarcomas, t(11;22)(q24;q12) in Ewing's sarcomas and peripheral neuroepitheliomas, and t(9;22)(q21-31;q11-12) in extraskeletal myxoid chondrosarcomas.

Bone Neoplasms↗

Primary malignant tumours of bone and soft tissue in the elderly.

AIMS: This study reports outcome, functional results and quality of life of 45 elderly patients with age over 70 after surgery for primary malignant bone and soft tissue tumours. METHODS: There were 24 primary malignant bone tumours and 21 soft tissue sarcomas. The most frequent diagnoses were: chondrosarcoma, malignant fibrous histiocytoma and liposarcoma. Local tumour resection with and without osteosynthesis, endoprostheses, and amputations had been performed for surgery. The patients were prospectively followed in a tumour register. RESULTS: Complication and revision rate, functional outcome using the Musculoskeletal Tumor Society score, 5-year survival rate, median survival time and quality of life according to the Life Satisfaction Index A and the global health and quality-of-life scale of the QLQ-C30 revealed results that are only slightly inferior to those reported in younger tumour patients. CONCLUSIONS: The results of this study generally justify even extensive tumour surgery in the elderly patient over 70 although outcomes are not quite as good as those reported for younger adults. However, especially in the old patient indications like general condition and comorbidity should be given due consideration before any decision is made on whether surgery should be performed and if so what surgical technique should be applied.

Age Factors↗

Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma).

A unique case of malignant peripheral nerve sheath tumor (MPNST) with perineurial cell differentiation occurring in a 63-year-old woman in a subcutis of the forearm is described. The tumor contained cellular and myxoid areas. The neoplastic cells were fusiform with distinct cell borders. They were arranged in storiform pattern and in wavy parallel cell cords in the cellular areas. Focally, a pleomorphism and mitotic activity (including atypical mitoses) similar to those of malignant fibrous histiocytoma were seen. The myxoid parts contained haphazardly oriented cells and scarce lipoblast-like multivacuolated cells mimicking a liposarcoma. In the differential diagnosis, myxoid liposarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma and low-grade fibromyxoid sarcoma were considered. Immunohistochemically, perineurial differentiation was indicated by the diffuse expression of epithelial membrane antigen and focal reactivity for CD34. The tumor was negative with antibodies to S-100 protein, Leu-7, CD68 (KP1), vimentin and cytokeratin AE1/AE3. Ultrastructure of tumor cells revealed features of MPNST. No recurrence occurred in the patient during 2 years follow up.

Antigens, CD34↗

Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location.

OBJECTIVE: The purpose of this study was to determine the relative prevalence, age at presentation, sex distribution, and skeletal distribution of malignant soft-tissue tumors and to ascertain the relative frequency of these tumors in specific anatomic locations and age groups among a population of patients in a large pathologic consultation service. MATERIALS AND METHODS: The computer diagnoses of 39,179 lesions occurring in 38,484 patients seen by soft-tissue pathologists at the Armed Forces Institute of Pathology during the 10-year period from January 1, 1980, to December 31, 1989, were retrospectively reviewed. All lesions were placed in one of 121 major categories in accordance with the classification system used by the World Health Organization and coded to one of 32 anatomic locations, such as hand, wrist, forearm, and so forth. Age and sex also were recorded. For purposes of analysis, all lesions were placed in one of 10 categories: hand and wrist, upper extremity, proximal limb girdle (axilla and shoulder), foot and ankle, lower extremity, hip and buttocks region, head and neck, trunk, retroperitoneum, and other lesions. The study group included 31,047 mesenchymal lesions, of which 12,370 were malignant. RESULTS: More than 80% of malignant tumors were classified into eight diagnostic categories: malignant fibrous histiocytoma (24%), liposarcoma (14%), leiomyosarcoma (8%), malignant schwannoma (6%), dermatofibrosarcoma protuberans (6%), synovial sarcoma (5%), fibrosarcoma (5%), and sarcoma, not classified further (12%). Approximately 79% of all malignant tumors were classified into five diagnoses for each age and location. With the distal upper extremity (hand and wrist) as an example, 50% of malignant lesions in the 16-25-year-old group were classified as epithelioid sarcoma (29%), malignant fibrous histiocytoma (13%), and synovial sarcoma (8%). For the same location but for children 5 years old or younger, almost 50% of malignant tumors were classified as infantile fibrosarcoma. CONCLUSION: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the location of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in establishing a suitably ordered differential diagnosis when a soft-tissue tumor has a nonspecific radiologic appearance.

Adolescent↗

Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis.

Focal myxoid change is a well-recognized feature of solitary fibrous tumor (SFT), but to date, predominantly myxoid examples of SFT have not been reported. We describe seven cases of SFT in which stromal myxoid change affected 50% or more of the tumor examined, thus obscuring typical diagnostic features. Patients ranged in age from 35 to 68 years old (median, 45 yr), with an equal sex distribution. Tumor locations included pleura, orbit, and periparotid subcutaneous tissue, as well as four cases in deep soft tissue (two in the abdominal wall and one each in the chest wall and thigh). Myxoid areas were identified grossly in four cases. Histologically, the lesions were composed of bland spindle cells disposed haphazardly or with a lacy or reticulated appearance in a myxoid, richly vascularized stroma These myxoid areas were punctuated by small cellular aggregates in four cases, and areas showing diagnostic features of SFT were present in five of seven primary excision specimens. Atypical features suggestive of malignancy were not present in any of the cases. Immunohistochemically, all of the seven cases stained positively for CD34 and CD99 (013), and all were negative for smooth muscle actin, desmin, S-100 protein, epithelial membrane antigen, and pan-keratin. There were no recurrences or metastases reported in four patients with limited follow-up (median duration, 19 mo). Recognition of this uncommon morphologic subset of SFT is important because of possible confusion, particularly in small biopsy specimens, with a variety of myxoid spindle cell neoplasms with different biologic potential. These include low-grade fibromyxoid sarcoma, myxoid synovial sarcoma, malignant peripheral nerve sheath tumor, low-grade myxofibrosarcoma, myxoid liposarcoma, myxoid spindle cell lipoma, myxoid neurofibroma, and so-called "hemangiopericytoma."

12E7 Antigen↗

[Hyperthermic perfusion with cytostatic drugs -- an adjuvant therapy in soft tissue sarcoma of the limbs (author's transl)].

Hyperthermic perfusion was used in 15 adult patients with soft tissue sarcoma of the limbs. Only patients were selected with prognostically unfavorable or recurrent tumors, which were excised with a limited safety margin. Three patients died from pulmonary metastases 16, 24 and 34 months after perfusion. In 2 patients a local recurrence developed. Ten patients are alive and free of recurrence. The follow-up period ranges from 7 - 47 months with a median of 30 months. These results encourage the further use of hyperthermic perfusion.

Antineoplastic Agents↗

Fusion of the FUS and BBF2H7 genes in low grade fibromyxoid sarcoma.

The FUS gene at 16p11 fuses with DDIT3 and ATF1 as the result of translocations with chromosome band 12q13 in myxoid liposarcoma and angiomatoid fibrous histiocytoma, respectively, and with ERG as the result of a t(16;21)(p11;q22) in acute myeloid leukemia. We here show that a t(7;16)(q33;p11) in two cases of low grade fibromyxoid sarcoma fuses the FUS gene to BBF2H7, a previously uncharacterized gene that is homologous to the Drosophila Bbf-2 gene. BBF2H7 spans more than 120 kbp genomic DNA, is composed of 12 exons and contains a 1560 bp open reading frame. It codes for a 519 amino acid protein that contains a basic DNA binding and leucine zipper dimerization (B-ZIP) motif, highly similar to that in the OASIS, CREB-H, CREB4 and CREB3 transcription factors, followed by a hydrophobic region predicted to be an alpha-helical transmembrane domain. Reverse transcription-polymerase chain reaction (RT-PCR), using FUS forward and BBF2H7 reverse primers, amplified FUS/BBF2H7 chimeric transcripts composed of the first five exons and part of exon 6 of FUS and part of exon 5 and exons 6-12 of BBF2H7. The FUS/BBF2H7 chimera codes for a protein containing the N-terminus of FUS and the B-ZIP domain and the C-terminus of BBF2H7.

Adolescent↗

The relative roles of surgery and radiotherapy in the management of soft tissue sarcomas of adults. A personal appraisal.

The literature of the last 25 years pertaining to the treatment of sarcomas of the soft somatic tissues by surgery and radiotherapy, both separately and in combination, has been reviewed. Surgery alone, if it is to be successful in an appreciable proportion of cases, must be radical. There is a growing body of evidence which suggests that less-than-radical surgery in combination with postoperative radiotherapy may give results equal or superior to those of radical surgery alone. There is need for a multicentre controlled clinical trial to compare the results of the two modes of treatment.

Adult↗

Myxoid liposarcoma of the spermatic cord: a case report and review of the literature.

Myxoid liposarcoma of the spermatic cord (MLSC) is a rare variance of spermatic sarcomas, with only 14 previous cases having been reported. Typically it presents during the seventh decade of life as a painless scrotal or inguinal mass. Although local recurrences are not uncommon, prognosis following complete tumour removal is good because metastases are rare. We describe one new case of a 24-year-old male with incidental MLSC found during inguinal hernia repair, and also review the existing literature.

Adult↗

[Giant retroperitoneal liposarcoma].

Liposarcoma is a malignancy of fat cells and is the most frequent soft tissue sarcoma localized in the retroperitoneum. It can reach substantial proportions. It is a slow-growing tumor, and the most frequent symptom is nonspecific abdominal pain and diffuse abdominal enlargement. Treatment is radical surgery and complete resection is essential for local control of the disease. We present a case of giant right retroperitoneal liposarcoma, which was well-encapsulated and could be completely excised. The patient is currently in follow up and at 2 years is disease-free.

Adult↗

Retroperitoneal sarcomas: a reappraisal of treatment.

Retroperitoneal sarcoma, regardless of the histopathologic grade of malignancy, has a high incidence of local recurrence that is usually fatal. Surgical resection is mandatory and resectability may be improved by a midline transabdominal approach. However, even with total tumor excision, recurrence is high. A preliminary study, combining resection and preoperative adriamycin, found to be effective for established disease, suggests potentially improved prognosis. Further clinical trials employing combined with adjuvant chemotherapy are warranted.

Adolescent↗