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PubMed1998

Soft tissue sarcomas: pattern diagnosis or entity?

Soft tissue sarcomas (STS) are a diverse and heterogeneous group of tumours. The sub-classification of these tumours is of importance for both prognosis and treatment. Classically, sub-categorization is based purely on histomorphological grounds, but as new techniques evolve, a more conclusive and accurate diagnosis can be made. This study describes the prevalence of soft tissue sarcomas in adults diagnosed at The Aga Khan University Hospital (AKUH) and the impact of immunohistochemistry (IHC) on the precise sub-categorization of these tumours. The study included 364 adults (age 16+) who were diagnosed as soft tissue sarcoma in the past six years (May 1991-July 1997) at the Histopathology lab of the AKUH. Where indicated, tumours were stained with a panel of antibodies using the PAP technique. Of these, 237 (65%) were male and 127 (35%) were female. The median age at which all sarcomas were diagnosed was 39.5 years. The most common site was the lower extremity (29%). The most frequently diagnosed sarcoma was leiomyosarcoma (13%), followed by malignant nerve sheath tumour (12%), rhabdomyosarcoma (10%) and liposarcoma (10%). Cases were further analyzed by dividing them into two groups, each group comprised of all sarcomas diagnosed during the specified period. In the period 1991-1994, only 16% of cases were further analyzed using IHC, while in the period 1995-1997, IHC was performed on 59% of cases. In the 1991-1994 group, a conclusive diagnosis was made in 57% of the cases and in the 1995-1997 group in 78%. A Chi-square test was performed, which proved that these results were statistically significant. Soft tissue sarcoma is one of the key areas in surgical pathology where immunohistochemistry plays an important role in both precise diagnosis and sub-categorization.

Abdominal Neoplasms↗
PubMed2003

Primary purely intradermal pleomorphic liposarcoma.

INTRODUCTION: Pleomorphic liposarcoma (PLPS) is a high-grade pleomorphic sarcoma, containing multivacuolated lipoblasts, which usually develops during late adult life. It usually occurs in the deep soft tissues and uncommonly arises in the subcutis, the dermis representing an exceedingly rare site of occurrence. METHODS: We describe a case of PLPS arising in the dorsal aspect of the nose of a 75-year-old woman. Preoperative duration was 9 months. RESULTS: The lesion was intradermal well-circumscribed, dome-shaped, measuring 1.2 cm. It fulfilled the histologic criteria for inclusion in the PLPS category. The tumor cells focally expressed calretinin. A review of the literature yielded four cases documented, to which we add the present report. All the patients were adults with a mean age of 67 (range 39-95) years, and three of five cases arose on the scalp. Local recurrence occurred in one patient, but no distant metastases or disease-related deaths were observed. CONCLUSION: PLPS very rarely arises in the dermis. In spite of high-grade morphology, the intradermal tumor shows a relatively favorable prognosis. Diagnostic consideration includes pleomorphic lipoma, clear cell atypical fibroxanthoma, balloon cell melanoma, and metastatic clear cell carcinoma of renal origin. Recognition of this distinctive and rare type of liposarcoma is essential to avoid under- or misdiagnosis and inappropriate treatment.

Aged↗
PubMed1998

Pre-operative core biopsy of soft-tissue tumours facilitates their surgical management.

BACKGROUND: Soft-tissue sarcomas are rare, and clinical differentiation of benign tumours from sarcomas is sometimes impossible. Further, the diagnosis of soft-tissue sarcomas may be unsuspected pre-operatively, and the presenting mass enucleated. While enucleation (excisional biopsy) is acceptable for benign lesions, it is inappropriate for sarcomas, because the opportunity for the most effective management resulting in both adequate local control and functional limb salvage surgery is compromised. A high rate of wound complications following open incisional biopsy may also compromise local treatment. Inappropriate siting of the incision for both incisional and excisional biopsies may adversely affect subsequent surgery and radiotherapy. METHODS: We therefore assessed the accuracy of core biopsy in the diagnosis of soft-tissue tumours, and planning of definitive surgery. All patients with primary soft-tissue tumours managed by two surgeons with a special interest in soft-tissue sarcomas since 1991 were reviewed. More than half (53%) were referred from other specialists. RESULTS: Of 45 cases, 37 (82%) were referred with the tumour intact, and of these 31 (84%) underwent core biopsy. The overall accuracy of core biopsy was 84%. The sensitivity was 94%, with 100% specificity. In most patients this allowed planning of definitive one-stage surgery (P < 0.005). Of the remaining five non-diagnostic cores, four were benign and one was a non-specific malignancy. CONCLUSIONS: Core biopsy has a high degree of accuracy in the diagnosis of soft-tissue tumours, particularly malignant lesions, and is not misleading. Core biopsy avoids the complications of open biopsy, and enables planning of one-stage surgery when used in combination with appropriate imaging.

Adult↗
PubMed1985

Limitations of computed tomography following excisional biopsy of soft tissue sarcomas.

Twenty-one patients were evaluated by computed tomography (CT) following complete or incomplete excisional biopsy of soft tissue sarcomas. Since the surgical margins were inadequate, additional treatment was required, and CT was intended to identify and delineate any residual tumor. Thirteen patients had no palpable mass in the operative area. In these, eleven CTs showed no tumor, but microscopic tumor was found in seven. The other two CTs showed masses, but both proved to be hematomas. Eight patients had palpable masses and seven proved to be residual tumor. Of these, two CTs failed to show the residual tumor. Five CTs correctly identified residual tumor, but two of them failed to show the entire tumor extent. Computed tomography did not correctly predict the presence or absence of microscopic residual tumor when there was no palpable mass, and was at least partly inaccurate in delineating residual tumor in four of eight patients with palpable masses.

Adolescent↗
PubMed1990

[Cytogenetics in pediatric solid tumors].

Chromosome abnormalities found in pediatric solid tumors include deletions, translocations, homogeneously staining regions (hsr)/double minutes (dms), and ploidy abnormalities. The discovery of a 13q14 deletion found in lymphocytes of patients with retinoblastoma and developmental delay has led to the cloning of the retinoblastoma gene. Likewise the discovery of an 11p13 deletion in lymphocytes of patients with Wilms' tumor and aniridia has led to the cloning of the Wilms' tumor gene. Chromosome deletions found in tumor cells are considered to play a role on the homologous deletion of cancer suppressor genes. Recently, various translocations have been found mostly in soft tissue sarcomas; i.e. t(11;22) in Ewing's sarcoma, t(2;13) in alveolar rhabdomyosarcoma, t(3;8) in pleomorphic adenoma, t(3;12) in lipoma, t(12;16) in liposarcoma, t(12;14) in leiomyosarcoma, and t(X;18) in synovial sarcoma. These translocations provide important information on the difficult diagnosis of soft tissue sarcomas, and on the selection of chemotherapy protocol. Tumor cells in advanced stage neuroblastomas often show hsr/dms, in which N-myc amplification occurs. While near triploidy was regularly found in early-stage neuroblastomas, near-diploidy or near-tetraploidy was usually found in advanced stage tumors. Among various prognostic factors, N-myc copy numbers and tumor cell ploidies had the largest influence on the prognosis of neuroblastoma patients. Cytogenetic and molecular genetic analyses on tumor cells are becoming increasingly important for the diagnosis of pediatric solid tumors, and the prediction of the patients' prognosis.

Adult↗
PubMed1983

[Significance of radiotherapy in the treatment of soft tissue sarcomas].

Postoperative photon- or electron irradiation has been well established in the treatment of soft tissue sarcomas. For further improvement of these results, neutron- or neutron-boost irradiation was introduced at several centres of radiation therapy. In this report, the 3.5 year results of 112 patients are analysed. The majority (62%) had T3-tumours (UICC classification, Geneva 1978). 58 patients presented with gross tumour at the beginning of radiotherapy, and 54 patients without clinical evidence of tumour. 39 patients (35%) had recurrent tumours. A highly statistical difference in local tumour control was found between the group of patients with gross tumour (47%) and the group of patients without clinical evidence of tumour (81%). A statistical difference in local control and survival was also found between the group of patients with T1-tumours (100%), T2 tumours (76.8%) and T3 tumours (44.5%), whereas no differences were found in terms of grading. The high complication rate of 25%, which was found after neutron therapy, was reduced to 7% after introduction of a neutron boost. The statistical significance of this treatment modality is presently being evaluated in a randomised EORTC trial. Adjuvant chemotherapy is recommended für all G3 tumours.

Adolescent↗
PubMedDate not supplied

The comparative role of immunohistochemistry and electron microscopy in the identification of myogenic differentiation in soft tissue pleomorphic sarcomas.

There is increasing evidence that histological classification of pleomorphic soft tissue sarcomas is prognostically useful, since a number of studies have provided evidence that myogenic differentiation is associated with a more aggressive clinical behavior. The aim of the current study was to analyze the role of electron microscopy in comparison with immunohistochemistry in the classification of soft tissue pleomorphic sarcomas. Thirty-nine pleomorphic sarcomas of the somatic soft tissues for which material for immunohistochemical and ultrastructural analysis was available were selected for this study. Cases were classified according to the criteria of the WHO classification of soft tissue tumors on the basis of the histologic appearance and of the results of immunohistochemical analysis, and then diagnoses were reconsidered at the light of the results of the ultrastructural analysis. The group of myogenic sarcomas included 13 leiomyosarcomas, 8 myofibrosarcomas, and 1 rhabdomyosarcoma, while the group of nonmyogenic sarcomas included 11 undifferentiated pleomorphic sarcomas/malignant fibrous histiocytomas (MFH), 4 myxofibrosarcomas, and 2 liposarcomas. Overall, there was a good concordance between immunohistochemistry and electron microscopy in recognizing myogenic differentiation in soft tissue pleomorphic sarcomas. Discrepancies included 1 case showing no immunoreactivity for muscle markers, which displayed ultrastructural features allowing reclassification as leiomyosarcoma, and 2 cases initially classified as undifferentiated pleomorphic sarcoma/MFH, which were reclassified as myofibrosarcomas after ultrastructural analysis. Ultrastructural analysis allowed the identification of pleomorphic sarcomas with myofibroblastic phenotype, a category that is not identifiable based on histologic and immunohistochemical profile. Notably, fibronexus junction was identified in tumor cells of 4 pleomorphic myofibrosarcomas, while 2 other lesions showed putative fibronexus junction structures, consisting of electron-dense straight fibrils adjacent to the cell surface, not clearly in continuity with cytoplasmic actin filaments. In conclusion, the results indicate that immunohistochemistry and electron microscopy can usefully complement each other in the classification of soft tissue pleomorphic sarcomas.

Actins↗
PubMed1997

[Dedifferentiated liposarcoma of the omentum simulating malignant peritoneal mesothelioma].

Liposarcoma of the omentum is a rare tumour which can simulate mesothelioma by naked eye and in histology as it was in the described case: A diffuse tumorous infiltration of parietal and visceral peritoneum was found in a male autopsy combined with two secondaries in the liver and one renal. The omentum was increased and quite infiltrated by the tumour. Single tumour foci consisted of structures reminding of a malignant neurogenous tumour, malignant fibrous histiocytoma and haemangiopericytoma. Some areas with a rather oval tumour cells with poor cohesivity reminded of a malignant lymphoma but were without any immunohistochemical positivity with markers proper for ML. A series of other tumours were to be eliminated in differential diagnostic consideration, i.e. sarcomatoid carcinoma, GIST, secondaries of a sarcomatoid variant of melanoma, malignant schwannoma, malignant fibrous histiocytoma, peritoneal leiomyomatosis. The prevalence of dedifferentiated structures can mostly veil the diagnosis of liposarcoma and immunohistochemical results are not always constant. A transition between well differentiated liposarcoma and its dedifferentiated structures are to be found in a single section for correct diagnosis. Prognosis of the dedifferentiated liposarcoma is presumed to be better than that of pleiomorphic sarcomas. The described patient was presented in terminal stage of disease and was monitored but for a month.

Aged↗
PubMed1982

Tourniquet infusion versus hyperthermic perfusion.

The tourniquet infusion method was compared with hyperthermic perfusion in canine limbs by using Adriamycin, actinomycin-D, and melphalan. Tourniquet infusion provided comparable tissue levels with Adriamycin and significantly higher levels with actinomycin-D and melphalan in the treated extremity than hyperthermic perfusion with the same drugs and dosages. Higher systemic leak was observed, more so with melphalan, with the tourniquet infusion method. Tourniquet infusion has caused complete regression of four malignant tumors involving extremities (one malignant melanoma, two Kaposi's sarcomas, one squamous cell carcinoma) and partial greater than 50% regression of nine tumors (three malignant melanomas, three squamous cell carcinomas, one malignant schwannoma, one malignant fibrohistiocytoma, one liposarcoma) followed by excision of residual tumor. Five patients with extremity sarcomas precluding adequate surgical margins were treated preoperatively with the this method. Longer follow-up is needed, as is a larger number of patients for a valid comparison of tourniquet infusion with hyperthermic perfusion.

Aged↗
PubMed2006

A novel germline mutation of MSH2 in a hereditary nonpolyposis colorectal cancer patient with liposarcoma.

BACKGROUND: One of the clinical features of hereditary nonpolyposis colorectal cancer (HNPCC) is a high incidence of multiple primary neoplasms arising in various organs including the gastrointestinal and genitourinary tracts. Among extracolonic tumors, a limited number of soft tissue sarcomas associated with HNPCC have been reported, and the mechanism underlying liposarcoma in HNPCC patients remains unclear. AIM: We herein report the case of a HNPCC patient with liposarcoma, with the goal of elucidating the involvement of a mismatch repair deficiency in the tumor. METHODS AND RESULTS: A 40-yr-old Japanese patient, who had a past history of adenocarcinoma of the rectum and transitional cell carcinoma of the urinary bladder, developed a liposarcoma in his left thigh. Although his family history did not fulfill the revised Amsterdam criteria, his blood sample was subjected to genetic testing. Direct sequencing of the genomic DNA from the blood identified an AT deletion at codon 677 in exon 13 of hMSH2, a pathogenic mutation that has not been reported before. The expression of MSH2 in the liposarcoma and rectal cancer of the patient was analyzed by immunohistochemistry, which revealed loss of MSH2 expression in the tumors. To investigate whether the loss of MSH2 was a common feature of liposarcoma, we examined the MSH2 expression in an additional two sporadic liposarcomas, both of which were stained with anti-MSH2 antibody. CONCLUSION: We identified a novel pathogenic germline mutation of MSH2 in an HNPCC patient. Since an immunohistochemical analysis showed no nuclear staining for MSH2 protein in the liposarcoma as well as the rectal cancer, the loss of wild-type MSH2 protein was thus considered to possibly play a role in the development of liposarcoma in HNPCC patients.

Adult↗
PubMed2005

Immunophenotypic characterization of high grade pleomorphic sarcomas: a demographic and immunohistochemical study in a major referral center of Pakistan.

OBJECTIVE: Immunophenotypic characterization of high grade (pleomorphic) sarcomas and determination of their frequency, mean/median age, sex preferences and common sites in Pakistani patients. METHODS: This study included 134 consecutive cases of high grade (pleomorphic) sarcomas diagnosed in adults above the age of 15 years in the section of histopathology at theAga Khan University Hospital, Karachi during a period of two years. These high grade (pleomorphic) sarcomas were immunophenotyped using a panel including antibodies against Vimentin, Desmin, Smooth muscle Actin, S 100, CD34, CD68 and Cytokeratin etc by indirect immunoperoxidase. RESULTS: Of the 134 cases which were characterized, 38.1% were pleomorphic leiomyosarcoma, followed by pleomorphic rhabdomyosarcoma 14.9%, Malignant Peripheral Nerve Sheath Tumour 9%, pleomorphic liposarcoma 3.7% and pleomorphic storiform Malignant Fibrous Histiocytoma 0.7%. Thirty three percent of cases could not be characterized further. Mean/ median age for Leiomyosarcoma was 50/50, for Rhabdomyosarcomas 33/22, for MPNST 42/41, for Liposarcoma 52/50 and for Malignant Fibrous Histiocytoma 46/46 respectively. The commonest site for leiomyosarcoma was lower limb (43%), for rhabdomyosarcoma head and neck (42%), for MPNSTthorax (36.4%) and for liposarcoma abdomen (50%). CONCLUSION: It was concluded that the most common pleomorphic sarcoma occurring in our adult population was Leiomyosarcoma, and that immunohistochemical stains are essential in most cases for further characterization of pleomorphic high grade sarcoma.

Adult↗
PubMedDate not supplied

Primary round cell liposarcoma of the omentum. A case report.

Liposarcomas are the most common of the malignant soft tissue tumors arising in the mediastinum and retroperitoneum. We have treated patients for round cell liposarcoma, an unusual histologic subtype, which is reported to be histologically mistaken for Ewing's sarcoma, lymphoma and other round cell neoplasms. The successful resection of a 2,300-g round cell liposarcoma of the omentum was recently performed at our hospital. Ultrastructural observations of this tumor are characterized by massive proliferation of the mitochondria in addition to the presence of lipid vacuoles in tumor cells. To our knowledge, no description of substantial mitochondrial proliferation in round cell liposarcoma has appeared previously in the literature; therefore, this is the first case report of histopathologic, cytopathologic and electron microscopic observations on this extremely rare tumor.

Female↗
PubMed1992

Gallium imaging in metastatic and recurrent soft-tissue sarcoma.

Fifty-six patients with metastatic or recurrent soft-tissue sarcoma were evaluated by 67Ga-citrate imaging. Prior to entry on the therapy protocol, 52/56 (93%) patients had true-positive 67Ga studies. Two of four patients with liposarcoma, one of twelve with leiomyosarcoma and one with an epithelioid sarcoma had false-negative studies; 89/105 disease sites (85%) were 67Ga positive, including 100% of pleural lesions, 94% in bone, 88% in the abdomen, 85% in soft tissue, 78% in lung parenchyma and 56% of liver metastases. There was significant association between 67Ga avidity and tumor grade with the exception of mesothelioma. No relationship was seen between 67Ga avidity and tumor cell type, disease site or lesion size. Following therapy, 67Ga correctly identified 11/12 sites of active disease in 8/9 patients. Mean pre- and post-therapy 67Ga avidity scores did not differ significantly. Gallium-67 appears to have an important role in the evaluation of patients presenting with either primary or metastatic soft-tissue sarcoma.

Adolescent↗
PubMed1977

Studies on adenovirus type 9-induced mammary fibroadenomas in rats and their malignant transformation.

After inoculating newborn W/Fu rats with adenovirus type 9, 27 of 27 females developed mammary fibroadenomas with a latency period of 14-25 weeks. No tumors were observed after inoculation with adenovirus type 5 or in males with the type 9 inoculation. After persistence of the tumors for 3-14 months, malignant transformation of the stroma resulted in different types of sarcoma in three rats: fibrosarcoma, round-cell liposarcoma, osteosarcoma and malignant mesenchymoma. In another animal the stroma of a fibroadenoma was highly cellular, suggesting a transition into fibrosarcoma. Malignant transformation of the epithelial component was not observed. Tumor cells contained adenovirus type 9-specific T-antigen, and rats with transplanted tumors were immunized to T-antigen. Mammary fibroadenomas without signs of malignant transformation developed in eight of nine female rats inoculated with adenovirus type 9 at an adult age. Neonatal thymectomy and total body x-irradiation neither significantly shortened the induction time of adenovirus 9-induced fibroadenomas nor increased the frequency of malignant transformation in females. One lipoma and one highly differentiated liposarcoma, however, appeared in two male rats. The results provide an example of the progression of a virus-induced benign tumor into a malignant neoplasm.

Adenofibroma↗