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Primary liposarcoma of the orbit is a rare tumour. There are very few cases of orbital liposarcoma reported in the literature, mostly of the myxoid variety. In this paper, the authors report the clinical presentation, histopathological features, results of diagnostic studies and management of a case of orbital low grade myxoliposarcoma with local recurrence, together with a review of the literature.
Ten consecutive patients with liposarcoma in the oral and maxillofacial region were analyzed retrospectively. Contrary to the majority of previous reviews, a female preponderance (two males and eight females) and younger incidence peak age (33 years) were found. Tumor sites were face (three), parotid (three), oral cavity (two), and mandible (two). Using a current histologic system, seven patients were classified as myxoid, and three as well-differentiated, round-cell, and pleomorphic lesions, respectively. Three patients died of recurrent disease 7 months, 20 months, and 4 years, respectively, after treatment; one patient died of metastatic liver and lung lesions and six patients remain free of disease (2 to 3 years). Clinical and histologic features, tumor behavior, treatment options, and patient prognosis are discussed and the literature is reviewed.
Myxoid liposarcomas harbor a unique and specific t(12;16)(q13,p11) chromosomal translocation. The breakpoint has recently been identified, and involvement of the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12 was demonstrated. We report a case of a 45-year-old woman who developed multiple malignant lipomatous tumors of unknown origin and myxoid/round cell histology at different locations. To examine the diagnostic potential of this translocation and to develop a hypothesis on the origin of the tumors, we used cytogenetic and molecular cytogenetic methods (reverse transcription polymerase chain reaction, RT-PCR). We identified a chimeric RNA transcript in the second recurrence in the thigh/groin, as well as in another tumor in the mediastinum, which has an additional sequence of 33 bp, known as fusion transcript type III. Cytogenetic analysis of another tumor in retroperitoneal space revealed a rare type of unbalanced translocation der(16)t(12;16). We hypothesize that these tumors are metastases rather than multicentric tumors. The detection of the chimeric message in the present case is not only useful for differential diagnosis, but also for analyzing the origin of multiple neoplasms.
A case of a male patient, 66 years old, who exhibited in a period of six years many multicentric and multifocal synchronic and metachronic myxoid liposarcomas is reported. The authors made a revision of cytogenetics and DNA alterations recently identified in this type of tumor. The chromosomic alterations are represented by the translocation t(12;16) (q13;p11) and trisomy of the chromosome 8. The genic analysis revealed the presence of altered restriction fragments due to highly specific and reproducible methylation differences. In conclusion the authors suggest that the presence of the multiple tumors of this type in the same patient could be due to a common etiologic factor, not yet known, as being the initiator of this systemic illness of the fat tissue.
We report a case of liposarcoma of the parotid gland. The tumor was initially controlled with radiation therapy, surgery, and chemotherapy, but the patient died in 24 months with widespread metastatic disease.
Head and neck liposarcoma is an extremely rare tumor. As with all rare lesions there is a void in the literature regarding tumor activity and treatment response. It is difficult to make rational treatment plans and advise patients as to probable treatment response and prognosis. To obtain as much information as possible from available data we have reviewed the world literature and reevaluated the descriptive histopathologic findings and treatment response of reported cases. Seventy-six cases have been reported since 1911. We added another case and then reclassified the previously reported lesions according to a current histopathologic system (well-differentiated, myxoid, round-cell, and pleomorphic lesions). We assessed tumor activity and treatment response by histopathologic tumor type. Paralleling tumor activity in other anatomic regions, all patients with myxoid and well-differentiated tumors did well; all were alive at the end of follow-up, eight of eight and 11 of 11, respectively (average follow-up, 5.7 and 4.8 years). This is compared with a 50% mortality rate in the round-cell and pleomorphic groups by the end of 2 years, (two of three and six of 11 patients alive, respectively). The likelihood of recurrence correlated with extent of tumor removal, and metastatic disease was identified almost exclusively with the tumor of the round-cell and pleomorphic variants. Surgical excision remains the primary treatment modality, while radiation and chemotherapy appear to be of limited utility.
It is well established that the majority of myxoid/round cell liposarcomas (LPS) are characterized by a reciprocal translocation t(12;16)(q13;p11) which at the molecular level results infusion of the CHOP and FUS/TLS genes. It is assumed that functional characterization of these genes may provide insight into the molecular pathogenesis of this tumour type. This study describes two new cases of myxoid/round cell LPS having a t(12;22). By reverse transcription-polymerase chain reaction (RT-PCR) it has been shown that this leads to fusion between the CHOP and EWS genes, thus indicating involvement of the EWS gene, at least occasionally, in yet another sarcoma type. Combining these two cases with two others which were recently similarly characterized at the molecular level, their clinicopathological features have been compared with cases having the more usual t(12;16). It was not possible to identify any clinical or pathological differences between these molecular genetic subsets. The relevance or significance of these gene fusion products in myxoid/round cell LPS remains to be determined.
The clinicopathological data of ten intramuscular myxomas (IMM) and three myxoid liposarcomas (MLS) are presented with emphasis on their ultrastructure. Electron microscopy of three of the IMM demonstrated the principal cell of the IMM to be similar to a fibroblast with prominent R.E.R., well-developed Golgi apparatus and cytoplasmic filaments. Intracytoplasmic lipid droplets were very rare. The matrix was composed of finely granular material, collagen fibers, fibrils and few capillaries. In contrast with the IMM, the cytoplasm of the MLS contained extensive lipid droplets. Capillaries were very abundant and in close proximity to the lipoblasts. The distinction between an IMM and MLS is usually clear-cut by light microscopy. Dissimilar ultrastructural features provide additional data to separate these two tumors. Ultrastructure confirmed Enzinger's impression that the cell of origin of the IMM is similar to a fibroblast with features that support the hypothesis that these cells are the source of production of the excessive amount of mucopolysaccharides in the matrix.
A case report of liposarcoma occurring in a man with probable hereditary multiple lipomata and metastasizing to bone marrow is presented. The neoplasm was studied by light and electron microscopy. The first evidence for metastatic disease was myelophthisic anemia. This unusual clinical occurrence is discussed briefly in conjunction with other neoplasms causing myelophthisis.
A human liposarcoma cell line COLO 222, derived from a primary tumor in a 62-year-old male, elaborates hyaluronic acid. COLO 222 is characterized on the basis of histochemical, ultramorphological, and cytogenetic properties, along with isozyme phenotype and cell products. A chromosome mode of 53 predominates and unique Giemsa-banded marker chromosomes are identified. An autochthonous lymphoid cell line, COLO 143v, was established after the addition of exogenous Epstein-Barr virus. Cytogenetic analysis of Colo 143v is consistent with a normal male karyotype. COLO 143v possesses B-cell characteristics. This autochthonous system had been used for immunological studies and cytotoxicity assays.
A clinicopathologic study of 48 cases of pleomorphic lipoma from the files of the Armed Forces Institute of Pathology reveals that this tumor occurs principally in males (83%) in the fifth to seventh decades (mean 57 years) and shows a predilection for the posterior neck, shoulder, and back. Typically, the lesion appears as a painless, circumscribed subcutaneous mass that, on gross examination, resembles an ordinary lipoma. However, microscopically, in contrast to the uniform appearance of the mature adipose tissue cells of an ordinary lipoma, this neoplasm is characterized by an intimate admixture of variably-sized fat cells, and bizarre, pleomorphic, multinucleated giant cells. Many of the latter cells show a distinctive floret-like arrangement of the nuclei and are associated with interlacing bundles of dense birefringent collagen. Despite this pleomorphic picture, which not infrequently leads to a misdiagnosis of liposarcoma, follow-up data obtained for 34 patients (median follow-up period of three years) establish the invariably benign clinical behavior of this unusual tumor.
Stimulated by the unexpected finding of gross abscesses in two cases of abdominal liposarcoma, we initiated a retrospective study to determine the incidence of this finding. Sixteen patients were analyzed, and correlations were made between histologic features and clinical course. Microscopic or macroscopic evidence of abscess was present in four cases. There appears to be an association between tumor necrosis or abscess and tumors with prognostically poor histology (e.g., pleomorphic tumors). Clinicians who treat patients with these tumors should be aware of the substantial risk of abscess and modify their therapy accordingly.
A pleomorphic liposarcoma arising in a site previously irradiated as a treatment for breast carcinoma is described. This case fulfills the criteria for a tumor to be radiation-induced. The tumor is localized with an irradiated area, there is a clear histologic distinction between the previous and current tumor, and there is a long latent interval.
In a series of 99 liposarcomas, the prognostic impact of the various diagnostic and therapeutic factors was analyzed. The analysis indicated that the tumor size, histologic subtype, and x-ray density are important prognostic factors. The prognosis is also influenced by operability and treatment modalities employed. Treatment strategy should be radical, irrespective of the actual prognostic factors.
This report describes an isolated case of cardiac metastasis of a myxoid liposarcoma that was successfully resected by surgery after clinical and ultrasound diagnosis. Although cardiac metastases are rarely diagnosed during patients' lifetimes, a search should be made for such lesions whenever cardiac disorders appear suddenly in cancer patients. Echocardiography appears to be one of the best investigative techniques for such purposes. Despite generally poor therapeutic results, surgical resection occasionally permits long periods of survival.
BACKGROUND: Surgery plays a dominant role in the initial and subsequent treatment of retroperitoneal liposarcoma (RPLS). This study was a review of outcomes of patients treated at the Royal Marsden Hospital. METHODS: Records of all patients who had surgery for RPLS since 1990 were reviewed, with particular attention to local recurrence and disease-specific survival. Patients with primary RPLS and those with recurrent RPLS, who had palliative surgery after a variable number of operations performed elsewhere, were considered separately. RESULTS: Seventy-two patients had surgery for primary RPLS, over half of whom underwent resection of a contiguous organ to achieve clearance. Follow-up of at least 12 months was available for 58 patients. Thirty-four patients had no evidence of recurrence after median follow-up of 26 (range 12-151) months. Low-grade tumour and macroscopic clearance of tumour were significantly associated with a reduced risk of local recurrence and improved survival. Forty-seven patients had palliative surgery for recurrent RPLS. Median survival from time of last operation to death was 27 (range 0-79) months. Follow-up was to a median of 68 (range 14-261) months. CONCLUSION: Patients with low-grade RPLS that has been completely resected at the initial operation have the most favourable prognosis. Palliative resection is worthwhile to treat troublesome symptoms of recurrence.
Fine-needle aspiration is now well established in the investigation of retroperitoneal masses. Many of these lesions are first detected in the course of radiological surveys of patients with known or suspected malignancy. It can be difficult to diagnose sarcomas on fine-needle aspiration material as many of these tumour do not show cytological features of malignancy. We describe two cases of metastatic liposarcoma confidently diagnosed on the basis of characteristic cytological features seen in material aspirated from metastatic adrenal tumours.
Well-differentiated liposarcomas (WDLs) are common soft-tissue tumors in adults. They are characterized by large marker chromosomes and/or ring chromosomes containing 12q-derived sequences in which MDM2 is consistently amplified. WDLs are subdivided into two subtypes according to their karyotype. Type D cells exhibit a near-diploid karyotype, with very few or no chromosome changes. Type H cells exhibit a near-tetraploid karyotype and many structural changes. Expression of P14ARF, MDM2, and TP53 proteins was assayed in the two WDL subtypes to establish whether distinct expression profiles correlated with cell ploidy. Although a transcriptionally functional TP53 was present in most tumors independent of their karyotype, type H cells were characterized by high levels of P14ARF and MDM2 proteins. Although amplified within similar chromosome markers in type D tumors, MDM2 did not appear to be overexpressed. In addition, it was present as a C-terminal truncated protein, indicative of alternatively spliced variants of MDM2 mRNA. As the existence of karyotypically distinct tumors could result from alterations of the mitotic machinery, we investigated the centrosome behavior in the two WDL subtypes. Centrosome amplification occurred in WDL tumors types H and D independent of their ploidy status. Moreover, no functional centrosome difference was found between the two tumor subtypes.