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Gliosarcoma with liposarcomatous differentiation: the new member of the lipid-containing brain tumors family.

Gliosarcoma is a rare malignant, biphasic brain tumor composed of glioblastoma multiforme and sarcomatous components. Various types of sarcomatous differentiation are described in this tumor: fibrosarcomatous, malignant fibrous histiocytoma-like, chondrosarcomatous and osteosarcomatous types. We report an extremely unusual variant of liposarcomatous differentiation in gliosarcoma in 72-year-old woman. Fat cells were presented by atypical multivacuolar and monovacuolar lipoblasts, stained positive for S100. p53 that was positive in both glial and mesenchymal cells of the tumor were negative in the lipoblasts. To the best of our knowledge, this is the first report in the literature of liposarcomatous differentiation in gliosarcoma.

Aged↗

Fine needle aspiration of pleomorphic giant-cell carcinoma of the pancreas. Case report with ultrastructural observations.

The fine needle aspirate in a case of pleomorphic giant-cell carcinoma of the pancreas, an unusual but highly malignant variant of ductal carcinoma of the pancreas, was characterized by bizarre tumor giant cells, "osteoclastlike" giant cells and abundant mitoses. The differential diagnostic possibilities include sarcoma (rhabdomyosarcoma, malignant fibrous histiocytoma and liposarcoma), melanoma, choriocarcinoma, metastatic giant-cell carcinoma of the lung and giant-cell tumor of the pancreas. A combination of clinical history, imaging findings and fine needle aspiration biopsy with transmission electron microscopy could lead to the appropriate diagnosis and help differentiate this entity from the other possible considerations.

Aged↗

[Biometric studies in the cytological diagnosis of soft tissue tumors].

Introducing of quantitative signs (the number of cells on the given area, size of cells, nuclei, optic and spectral characteristics) makes the cytologic diagnosis much more objective. Karyometric studies conducted by the author (the determination of nuclei volume) on cytological smears, obtained from 16 patients with soft tissue tumors, indicated some difference in nuclei size of various histotypes. The studies have evidenced that the method of karyometry associated with cytophotometry will offer the pre-conditions for using karyometry in the practice of pathocytologists and facilitate the finding of algorithms for computing analysis of soft tissue neoplasms.

Cell Nucleus↗

Computed tomography of musculoskeletal tumors.

Modern chemotherapy has resulted in increased survival and even cure in many patients with malignant bone and soft tissue tumors. As a results, whereas amputation was commonly practiced in the past, many centers now use limb preservation surgery in selected patients. This technique requires accurate preoperative assessment of tumor location, extent and relationships, and the role of different imaging techniques, including conventional radiography, CT, radionuclide scanning, sonography and angiography in evaluating these parameters is discussed. CT provides the most accurate technique currently available for assessing the local extent of musculoskeletal tumors and has thus had a significant effect on their management. An algorithmic approach to the evaluation of musculoskeletal tumors is proposed.

Angiography↗

FUS/TLS-CHOP chimeric transcripts in liposarcoma tissues.

Myxoid liposarcoma and malignant fibrous histiocytoma (MFH) are common soft tissue sarcomas of adulthood. Histopathologically they often show intratumor heterogeneity. In some cases, differential diagnosis of liposarcoma and MFH is difficult. It has been reported that myxoid liposarcomas are characterized by chromosomal translocation t (12; 16) (q13; p11), and that this results in two types (type I and type II) of FUS/TLS-CHOP fusion transcripts. In this study, the FUS/TLS-CHOP chimeric transcripts in seven malignant soft tissue tumors of Asian patients were analyzed by reverse transcription-polymerase chain reaction, DNA blot hybridization and nucleotide sequencing. One myxoid liposarcoma and two round cell liposarcomas possessed a chimeric transcript whose fusion point was the same as that of the type I fusion transcript reported previously for myxoid liposarcoma. We were thus able to detect the type I FUS/TLS-CHOP fusion transcript in clinical specimens of liposarcoma from Asian patients, including the first examples of round cell liposarcoma. These results suggest that the detection of FUS/TLS-CHOP chimeric transcripts or chimeric genes can be used as a diagnostic tool for the pathological diagnosis of liposarcomas.

Adult↗

Dedifferentiated liposarcoma of the pyriform sinus: report of a case and review of the literature.

Laryngeal and hypopharyngeal liposarcomas are extraordinarily infrequent tumors. To the best of our knowledge there are fewer than 40 well-documented cases reported to date. Almost all of them are well-differentiated liposarcomas, with only 2 laryngeal-hypopharyngeal dedifferentiated liposarcomas. Dedifferentiated liposarcoma is defined as a well-differentiated liposarcoma with areas of high-grade spindle cell nonlipogenic sarcoma. The well-differentiated areas may be of a lipoma-like, sclerosing, or mixed type, and the dedifferentiated areas most frequently are of malignant fibrous hystiocytoma-like type. Despite its commonly pleomorphic histology, dedifferentiated liposarcoma does not behave as aggressively as most pleomorphic sarcomas of adulthood; however, it has the capacity to metastasize, in contrast to its well-differentiated counterpart. We present a case of dedifferentiated liposarcoma arising in the pyriform sinus, an event only twice reported previously in the literature.

Aged↗

Nonosseous sarcomas in a military hospital.

This is a retrospective review of 67 patients with soft tissue or visceral sarcomas treated during 1973-1982. There were 40 males and 27 females. The mean age was 36 with a range of 1 to 77 years of age. The most common histologic subtypes were rhabdomyosarcoma (19%), malignant fibrohistiocytoma (13%), fibrosarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans (12% each), and liposarcoma (10%). Among the 42 soft tissue sarcomas 33% occurred in the trunk, 35% and 21% in the lower and upper extremities, and 10% in the head and neck region. Among the 25 visceral sarcomas, 16% were in the thorax, 56% involved abdominal or retroperitoneal organs, and 28% were in the head and neck region. Overall, 33 of the 54 patients with early sarcomas were treated by surgical resection alone while 21 others had postoperative adjuvant radiotherapy and/or chemotherapy. Factors influencing survival are presented and patterns of metastasis discussed. Overall, 45% of the patients showed metastatic disease either at diagnosis (15%) or during the follow-up period (30%). The lung was involved in two-thirds of the cases and was the most common site of spread. In patients with rhabdomyosarcoma and malignant fibrohistiocytoma, regional lymph node metastasis was noted in 36% and 17%, respectively.

Adolescent↗

A novel zinc finger gene is fused to EWS in small round cell tumor.

Ewing sarcoma family of tumors share recurrent translocations that fuse EWS from 22q12 to five different members of transcription factors namely FLI-1, ERG, ETV1, E1AF and FEV. Different classes of DNA binding proteins, ATF1, WT1 and CHOP are fused to EWS generating distinct tumor phenotypes: clear cell sarcoma, desmoplastic small round cell tumor, and myxoid liposarcoma, respectively. We have cloned a novel gene located at 22q12 fused to EWS by a submicroscopic inversion of 22q in a small round cell sarcoma showing a translocation (t(1;22)(p36.1;q12). The gene, designated ZSG (Zinc finger Sarcoma Gene), is a putative Cys2-His2 zinc finger protein which contains a POZ transcriptional repressor-like domain at the N-terminus. The rearrangement involves intron 8 of EWS and exon 1 of ZSG creating a chimeric sequence containing the transactivation domain of EWS fused to zinc finger domain of ZSG. This product lacks the transcriptional repressor domain at the N-terminus of ZSG. A rearrangement of the second ZSG allele was also found in tumor cells. This is the first example of an intra-chromosomal rearrangement of chromosome 22, undetectable by cytogenetics, activating EWS in soft tissue sarcoma.

Adolescent↗

Lymphographic evaluation in bone and soft tissue sarcomas.

A group of 79 patients with soft tissue sarcomas and 46 with primary malignant bone tumors underwent lymphangiography as part of the initial diagnostic workup. In the group of soft tissue sarcomas, the overall incidence of metastases was 22 of 79 (28%). According to the site of origin, the highest incidence was found in tumors originating from the buttocks (57%), followed by those from the inferior limbs (31%). Considering the histology, the incidence ranges from 50% in anaplastic sarcoma, to 43% in rhabdomyosarcoma, to 23% for liposarcoma and fibrosarcoma. The incidence of lymphographically proven metastases in bone tumors was 8 to 46 (17%), with 1 out of 4 in reticulum cell sarcoma, 1/1 in chordoma, and 22% in Ewing's sarcoma. A radiographic/histologic correlation on the lymph nodes was obtained in 19/79 (24%) soft tissue sarcomas and in 4/46 (9%) bone tumors. In the 12 radiographically negative and 11 radiographically positive cases, this correlation was always correct. The results of this study suggest a larger use of lymphography in these tumors.

Bone Neoplasms↗

[Primary liposarcoma of the left ventricle. Apropos of a case and review of the literature].

Primary malignant myocardial tumours are rare and essentially sarcomas. The authors report a case of primary left ventricular liposarcoma which is the 18th reported case. The presenting signs were of cardiac failure. Metastases are common by the time of diagnosis. Surgical ablation, though rarely complete because of its myocardial localisation, is justified for precise histological diagnosis. The prognosis of these lesions is poor. Complementary treatment is rarely used. However, it should be proposed as surgery alone has been shown to have limited curative applications.

Adult↗

Well differentiated "lipoma-like" liposarcoma of the sigmoid mesocolon and multiple lipomatosis of the rectosigmoid colon. Report of a case.

Liposarcoma is the second most common soft tissue sarcoma in adults. These neoplasms take their origin from primitive mesenchymal cells and are rarely encountered in fat rich areas, such as subcutaneous tissue and/or the subserosa of the intestinal tract which, on the contrary, are the two most common sites of lipomas. The two major locations of liposarcomas are the extremities and the retroperitoneum followed with much less frequency by the inguinal region. Other sites are uncommon, particularly the mesentery (9 cases to date in the literature) and, even more so, the mesocolon (only 3 cases of primary sarcoma of the mesocolon reported to date). This paper reports on the case of a well differentiated "lipoma-like" liposarcoma of the sigmoid mesocolon, associated with multiple lipomatosis of the recto-sigmoid colon in a 75 year-old female patient. Surgical treatment consisted of a trans-anal extra-peritoneal anterior resection by CEEA 28 stapler under endoscopic vision. The patient has been followed up for the last 2 years and is still disease-free and well. The peculiarity of the case consists in the contemporaneous presence in close contiguity of two different rare neoplasms whose association is not yet known.

Adult↗

Fine-needle aspiration smears from lipomatous hemangiopericytoma need not be confused with myxoid liposarcoma.

Lipomatous hemangiopericytoma (LHPC) is a rare benign soft tissue tumor that may mimic soft tissue sarcoma clinically. Despite the fact that LHPC shares most of the histologic features with solitary fibrous tumor, it may be occasionally misdiagnosed as myxoid liposarcoma or some other type of spindle-cell sarcoma. While some studies have addressed the histological features and origin of LHPC, no reports of cytological findings have been published. The author describes the cytological features of LHPC in two patients with correlations to histological and clinical findings. One of aspirates was misdiagnosed as being myxoid liposarcoma, while the other one was correctly diagnosed by a combination of FNAB and core needle biopsy (CNB). Cytological evaluation of LHPC can be problematic due to its rarity and to its similarity with other spindle-cell or fatty tumors. Correct diagnosis requires an awareness of cytologic features of this rare tumor and the use of ancillary studies.

Adipocytes↗

The value of fine-needle aspiration biopsy in the differential diagnosis of adult myxoid sarcoma.

BACKGROUND: The usefulness of fine-needle aspiration biopsy (FNAB) for the histologic subtyping of specific sarcomas still is somewhat controversial but is becoming increasingly popular in the U.S. METHODS: To determine the accuracy and usefulness of FNAB in the differential diagnosis of myxoid sarcoma, the authors retrospectively reviewed 18 FNAB specimens (16 primary tumors, 1 local recurrence, and 1 metastasis) in 18 patients. The study sample included myxoid/round cell liposarcoma in six patients, myxofibrosarcoma in six patients, myxoid chondrosarcoma in five patients, and myxoid leiomyosarcoma in one patient. RESULTS: All but one tumor were recognized correctly as malignant. With regard to primary tumors, a specific cytologic diagnosis was rendered in 13 of 16 patients (81%). Problematic areas included the diagnosis of high grade myxofibrosarcoma with minimal amounts of myxoid stroma, myxoid liposarcoma with a predominant round cell component, and the single case of myxoid leiomyosarcoma. CONCLUSIONS: FNAB represents a valuable diagnostic tool for the differential diagnosis of myxoid sarcoma, especially myxofibrosarcoma, low grade myxoid liposarcoma, and myxoid chondrosarcoma. Due to its prognostic and therapeutic significance, the presence of a predominant round cell component in myxoid liposarcoma should be documented adequately. Other sarcomas (e.g., leiomyosarcoma) rarely may exhibit a prominent myxoid stroma and therefore should be considered in the differential diagnosis of adult myxoid sarcoma.

Adult↗