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Retroperitoneal sarcoma can occur in the female pelvis and be confused with other pelvic masses. Its diagnosis depends on an awareness of its occurrence. Aggressive surgery to completely excise the tumor determines survival.
The authors report a case of well differentiated paratesticular liposarcoma in a 41-year-old patient. This is a rare tumour (about one hundred cases have been reported in the literature), which essentially arises from the spermatic cord. Clinical and radiological signs are nonspecific and the diagnosis is generally based on histological examination of the operative specimen. Due to the histological similarities, all specimens of benign lipoma must be examined for the presence of well differentiated liposarcoma. Treatment consists of transinguinal radical orchidectomy, sometimes with resection of adjacent structures. The prognosis is generally better than that of other paratesticular sarcomas. Adjuvant radiotherapy may be indicated in locally advanced masses or in the case of incomplete resection.
A number of human cell lines derived from well-differentiated, myxoid/round cell, or pleomorphic liposarcoma have been described. To our knowledge, however, no human cell line established from dedifferentiated liposarcoma has been reported. In this study, we established a new human cell line, FU-DDLS-1, which originated from a dedifferentiated liposarcoma arising in the retroperitoneum of a 61-year-old man. This cell line was characterized by immunocytochemistry, conventional banding analysis, fluorescence in situ hybridization with chromosome painting probe, and comparative genomic hybridization (CGH). FU-DDLS-1 cells were spindle or polygonal shaped and possessed oval nuclei and slender cytoplasmic processes. The cultured cells were successfully maintained in vitro for over 90 passages over more than 30 months. The histologic features of heterotransplanted tumors in severe combined immunodeficiency mice were essentially the same as those of the original nonlipogenic sarcoma resembling a malignant fibrous histiocytoma. Both in vitro and in vivo, the cells exhibited immunopositive reaction for mdm2 and p53 proteins. Cytogenetically, FU-DDLS-1 displayed a hypertetraploid karyotype with giant marker chromosomes composed partly of chromosome 12 material. In addition, CGH analysis demonstrated that DNA sequence copy number changes including a gain of 12q12-q21 detected in FU-DDLS-1 were essentially the same as those in the original sarcoma. The FU-DDLS-1 cell line, which exhibits the unique conventional and molecular cytogenetic characteristics of dedifferentiated liposarcoma, should be a particularly useful model for studying the molecular pathogenesis of human dedifferentiated liposarcoma.
Spindle cells seen in fine-needle aspiration biopsy (FNAB) of the mediastinal lesions can be a component of a wide variety of benign and malignant conditions. Few of these conditions, however, are described in the FNA cytopathology literature. This review discusses the cytopathologic features, differential diagnoses, and potential pitfalls of a variety of lesions with a significant component of spindle cells encountered in mediastinal FNAB. The cytopathology files from four institutions were searched for cases of mediastinal FNAB containing a spindle-cell component that was a key or predominant cytologic feature of the diagnostic specimen. The cytomorphologic features of these cases were analyzed, and their differential features are discussed. Of 196 mediastinal FNABs, 22 (11%) were lesions with significant spindle-cell component: granulomatous inflammation (four); benign nerve sheath tumor (four); thymic cyst (two); spindle-cell thymoma (two); large-cell non-Hodgkin's lymphoma with sclerosis (two); nodular sclerosing Hodgkin's disease (two); liposarcoma (two); spindle-cell squamous carcinoma possibly arising in a teratoma (one); unspecified high-grade sarcoma (one); spindle-cell malignant melanoma (one); and nonspecific fibrous tissue (one). The cytologic features of each lesion were analyzed as an aid for accurate classification. These findings were correlated with radiologic and clinical information when available. The value of ancillary studies performed on aspirated material in selected cases was also reviewed. FNA of mediastinal lesions with significant spindle-cell morphology represents an infrequent and heterogeneous group of entities that may pose significant diagnostic challenges. This review presents the salient cytopathologic features of various spindle-cell lesions of the mediastinum with particular emphasis on differential diagnosis and pitfalls. The pathologist must use caution when interpreting these lesions and ancillary studies may be of significant value in selected cases.
This article is a survey of actual aspects. With regard to frequency, the malignant fibrous histocytoma comes first, followed by lipo- and fibrosarcoma, synovial sarcoma, malignant schwannoma, malignant tumours proceeding from arteries and veins and the unstriated musculature. Staging and grading of these tumours are difficult. Until now their overall TNM-classification was not possible due to insufficient hard criteria.
Over a 10 year period, between 1974-1984, 257 adult cases of tissue sarcoma have been evaluated in the Department of Medical Oncology, Christie Hospital, Manchester. At registration locally advanced or metastatic diseases was present in 162 (63%). The male/female ratio was 1.5:1 and median age 54 years (range 14-85). The commonest sites were lower limb (33%), visceral (21%), trunk (13%), retroperitoneum (12%) and upper limb (10%). Leiomyosarcoma (27%), liposarcoma (14%) malignant fibrous histiocytoma (10%) and neuro plus fibrosarcomas (15%) were the most frequent histological subtypes. A high proportion of uterine sarcomas (17%) is a point of distinction from many other series. Histological grade was specified in 72% of cases and the distribution (Grade I--27%; II--6%; III--67%) reflected a referral bias towards advanced disease. Local resection of the primary tumour was performed in 76% of cases. In many instances this only amounted to 'shelling out' and true compartmental excisions were rare. Amputation was performed in 31% of patients with limb sarcomas. Ninety-eight patients (38%) had experienced one or more local recurrences prior to referral and the overall local recurrence rate was 56%. Suitable patients (78%) received chemotherapy, 50% entering multicentre trials in collaboration with the EORTC. The commonest regime used in patients with advanced disease was CYVADIC which produced an overall response rate of 37%. Ifosfamide, used as a single agent in 16 patients, induced 3CR and 5PR for an overall response rate of 50%. When used in combination with MTX and VADIC, there was no difference in response rate, but numbers in these pilot studies were small. Seventeen high risk patients received adjuvant chemotherapy with VAC, but the results (11 relapses) were disappointing. An EORTC trial, comparing adjuvant CYVADIC chemotherapy with control has accrued 307 patients, 49 of these from the Christie Hospital. Preliminary results within this centre - 13/25 relapses in the control arm, 5/23 in the chemotherapy arm-suggest an advantage for chemotherapy but the data are statistically not significant. Post-operative radical radiotherapy after resection of the primary tumour or local recurrence was performed in 51 patients, with local control in 65% of cases, although metastases developed in 41%. At the time of analysis (1st April 1984) 98 (38%) were alive, of whom 72 showed no evidence of disease and 52 had never relapsed. Malignant disease was the cause of death in 92%. Overall survival was not influenced by sex, but patients less than 40 years of age fared significantly better (P less than 0.001).(ABSTRACT TRUNCATED AT 400 WORDS)
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Monobloc soft-part resections have been carried out with excision of segments of the illac and femoral vessels in seven patients with cancer in the lower extremity. Amputation would have been necessary otherwise. Ischemic loss of the extremity did not occur. In four of the earlier patients, vascular reconstruction was not performed, and postoperative lymphedema was a major complication. In the recently treated three patients, complete reconstitution of the circulation by simultaneous arterial and venous grafting permitted smooth postoperative recovery and absence of notable edema. Creation of a distal arteriovenous fistula appears essential to maintain venous patency. Six of the seven patients are alive at six months, 1, 3, 4, 7, and 18 years, respectively. One patient died of pulmonary metastases at three years after operation. Two patients developed local recurrences requiring limb disarticulation, both in the earlier group in which excision and grafting of both artery and vein was not done. It is concluded that excision and simultaneous reconstruction of major blood vessels can extend the scope of soft-part resections for cancer of the extremities, offering opportunities for preservation of limbs that would otherwise require amputation for control of disease.
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A spontaneous non-immunogenic transplantable liposarcoma in BN rats was found to be sensitive to the antitumor effects of rat fibroblast interferon (RIF) when it was administered from the day of tumor implantation onwards. Treatment with RIF starting at 7 days after implantation was not effective. Tumor growth was markedly inhibited by cyclophosphamide (Cyclo). At the time and dose schedules used, Cyclo was more effective than RIF. When the growth of the tumor was inhibited by Cyclo, subsequent treatment with RIF did not lead to an additional retardation of tumor growth. Administration of RIF interfered with the beneficial effect of Cyclo when both agents were given concomitantly. RIF and Cyclo gave similar results to those obtained with RIF alone and these were inferior to those obtained with Cyclo alone.
The transcription factor CHOP is involved in the regulation of the cell division cycle and the control of programmed cell death in response to cellular stress. CHOP expression has been linked with several forms of cancer. A reciprocal translocation between the CHOP and TLS RNA-binding protein gene results in myxoid liposarcoma and amplifications of the CHOP gene are associated with solid tumors including several types of sarcomas. Here we report the mapping of the methionyl tRNA synthetase (MetRS) gene to the identical 12q13 locus where the CHOP gene had previously been mapped. PCR analysis demonstrates a tail-to-tail overlap of both genes over a 55-bp region. As a result the two mRNAs share a 3' UTR complementary sequence allowing an in vivo interaction between the two mRNAs. An AU-rich regulatory element (ARE) known to control mRNA stability resides in the overlapping sequence. To test for functional significance of the ARE a luciferase reporter plasmid containing the 3'UTR of CHOP was constructed. Transfection experiments in NIH-3T3 cells show that CHOP 3'UTR confers a significantly lower activity than a control reporter or a reporter in which the region overlapping the MetRS mRNA is deleted. The conservation of this overlapping of the CHOP and MetRS genes and the role of their complementary sequence in the control of mRNA stability suggest the existence of a functional link between the expression of these two genes.
Sclerosing epithelioid fibrosarcoma (SEF) is a recently described entity. It is a low-grade sarcoma that occurs primarily in the deep soft tissues of the extremities of adults. It may histologically simulate benign lesions such as fibroma and myxoma or malignancies such as sclerosing carcinoma and lymphoma, extraskeletal myxoid chondrosarcoma, clear cell sarcoma of tendons and aponeuroses, and synovial sarcoma, depending on the lesion's cellularity, degree of fibrosis, and amount of myxoid matrix. There are no previously published cytogenetic studies of this tumor. We found the karyotype 40-45,XY,add(9)(p13),add(10)(p11),-12,-13,-18,add(18)(q11),add(20)(q11) in a SEF of a 14-year-old boy, by using chromosome banding. Fluorescence in situ hybridization showed that both the add(10) and the add(18) contained amplified sequences from 12q13 and 12q15, including the HMGIC gene. Chromosome 18 material was present in the add(9) and terminally in the add(10). The karyotype of this case indicates that SEF is unrelated to extraskeletal myxoid chondrosarcoma, clear cell sarcoma, and synovial sarcoma. When compared with the findings in other soft tissue tumors such as well-differentiated liposarcoma and low-grade malignant fibrous histiocytoma, the chromosome banding and in situ hybridization data add support to the notion that SEF is a relatively low grade variant of fibrosarcoma.
Soft tissue tumors of seven patients were imaged with magnetic resonance imaging (MRI) prior to operative resection. In four cases, gross surgical specimen biopsy sites were broadly selected from areas of diverse signal intensity. A subsequently developed "grid analysis" technique, used in three additional patients, better localized differing zonal intensities within individual gross specimens. Further investigation of this technique is warranted, since MR images, when analyzed by means of a detailed grid technique, appear to enhance histologic diagnostic accuracy. Examination of larger segments of inhomogeneous soft tissue tumors is facilitated, while pathologists' sampling time is diminished.
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Computed tomography was performed on 52 patients with soft-tissue tumors, 36 malignant and 16 benign. Major advantages with CT include the possibility of differentiating between lipoma and other tumors and the ability to demonstrate the transverse location of a lesion (intra- or extracompartmental). It was not possible to relate attenuation values to histologic type or grade of malignancy. CT was found superior to conventional radiography in demonstrating additional bony destruction of the pelvis and spine. The risk of overestimating the size of high grade malignant tumors because of accompanying edema is discussed. CT should precede angiography in the investigation of soft-tissue tumors, and angiography may primarily be reserved for those lesions where vascular relationships are not adequately demonstrated by CT.
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