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A primary liposarcoma of the lung occurred in an 18 year old girl, the first to be reported in anyone under 40. Though rare, intrathoracic liposarcoma should be included in the differential diagnosis of pleural effusion in younger patients.
An elderly woman presented with dyspnoea secondary to extensive mediastinal invasion by a primary well differentiated liposarcoma of the mediastinum. Five years after partial resection and postoperative radiotherapy (45 Gy) she is alive and well. To our knowledge, long term survival has not previously been reported for a non-encapsulated mediastinal liposarcoma treated by incomplete resection and subsequent radiation therapy.
Liposarcomas of the gastrointestinal tract are exceedingly rare. Only nine cases of esophageal involvement have been described. A 68-year-old woman presented with an episode of vomiting followed by extrusion of a polypoid mass from the mouth. This 10th case of esophageal liposarcoma is the first in the literature to report a recurrence 25 years after the first episode.
A case of paratesticular liposarcoma in a transplanted patient is presented. Liposarcomas of the urogenital system are very rare tumors and have never before been reported in an immunosuppressed transplanted patient. We present a case of considerable clinical interest, our therapeutic approach and the final outcome.
Paratesticular masses can pose difficult diagnostic and therapeutic problems to the physician. We report a rare case of paratesticular liposarcoma with the clinical symptomatology of an inguinal hernia. The treatment was surgical and included radical orchiectomy and wide excision of the tumor mass to the macroscopically healthy margins. The patient is well and with no evidence of recurrence 1 year after the operation. We review the literature, and discuss the role of radical orchiectomy, radiation, and chemotherapy in the treatment of paratesticular liposarcomas.
AIMS AND BACKGROUND: The aim of this study was to describe a case of primary orbital liposarcoma in Li-Fraumeni syndrome. METHODS AND STUDY DESIGN: In July 1998 a 20-year-old woman with a histological diagnosis of orbital myxoid liposarcoma underwent surgical treatment in our department. Since the patient's family pedigree met the clinical criteria for the diagnosis of LFS, molecular analysis was performed, which resulted in a molecular profile consistent with Li-Fraumeni syndrome. RESULTS: The patient underwent orbital exenteration extended to the upper eyelid; surgical reconstructive steps were performed to permit placement of an orbital prosthesis. Two years after primary surgery the patient underwent a quadrantectomy with lymphadenectomy of the right axilla because of the presence of a nodule of 1.5 cm in diameter in the upper-lateral quadrant of the right breast. One year after the last surgery, the patient is disease free. CONCLUSION: The diagnosis of an orbital malignancy in a young patient with a family history of cancer should suggest the presence of an underlying genetic disorder like LFS; with molecular analysis we can now determine the genetic disorder and the exact location of the mutation, and also obtain important prognostic data using specific cellular markers. More prognostic information increases the chances of adequate personalized treatment.
Liposarcoma is a soft tissue tumor usually observed in adults. Although occasional cases have been reported in childhood, it is virtually unknown in infants and small children. Our experience documents a case of liposarcoma in a 6-year-old girl, an extremely rare occurrence in this age-group. The patient underwent wide local excision followed by chemotherapy. Histologically, the tumor was a myxoid type. Differential diagnosis and problems concerning prognosis and therapy of this tumor in children are discussed.
Nuclear receptors for steroid hormones, thyroid hormone, retinoids, and vitamin D are thought to mediate their transcriptional effects in concert with coregulator proteins that modulate receptor interactions with components of the basal transcription complex. In an effort to identify potential coregulators, receptor fusions with glutathione-S-transferase were used to isolate proteins in nuclear extracts capable of binding nuclear hormone receptors. Glutathione-S-transferase fusions with mouse retinoid X receptor-alpha enabled the selective isolation of a 65-kDa protein (p65) from nuclear extracts of rat and human cells. Binding of p65 to mouse retinoid X receptor-alpha was centered around the DNA-binding domain. p65 also bound regions encompassing the DNA-binding domain in estrogen, thyroid hormone, and glucocorticoid receptors. p65 was identified as TLS (translocated-in-liposarcoma), a recently identified member of the RNP family of nuclear RNA-binding proteins whose members are thought to function in RNA processing. The N-terminal half of TLS bound to thyroid hormone receptor with high affinity while the receptor was bound to appropriate DNA target sites. Functional studies indicated that the N-terminal half of TLS can interact with thyroid hormone receptor in vivo. TLS was originally discovered as part of a fusion protein arising from a chromosomal translocation causing human myxoid liposarcomas. TLS contains a potent transactivation domain whose translocation-induced fusion with a DNA-binding protein (CHOP) yields a powerful transforming oncogene and transcription factor. The transactivation and RNA-binding properties of TLS and the nature of its interaction with nuclear receptors suggest a novel role in nuclear receptor function.
A review of the literature of time-dose relationships of liposarcoma is given. The radiotherapeutic data on 15 measurable liposarcoma sites, together with 45 sites collected from the literature, are subjected to a Strandqvist plot analysis using the modified Ellis' formula TSD = D.T-alpha. The recovery coefficient alpha is thought to be rather high, of the order alpha = 0.5. This suggests that the use of a few large fractions in therapy may be beneficial.
Metastatic liposarcoma of the thyroid gland is exceptionally rare. A case of pleomorphic liposarcoma, which manifested as a soft tissue lump in the neck, is described in a 30-year-old woman. Fine needle aspiration cytology permitted prompt diagnosis. The ultrasound and CT appearance of this type of thyroid metastasis is described which has not been previously reported in the literature.
A case of lipoblastic liposarcoma of the neck is presented. The case is that of a 5-year-old female who underwent a modified neck dissection with a good result. A review of the literature reveals that this is an extremely rare tumor of the neck. It is usually found in the lower extremities, predominantly in adult males. Surgery is the treatment of choice. Lipoblastic liposarcoma is an extremely rare tumor when it occurs in the pediatric neck. Hudson and Saunders in their studies have shown that only 3 patients with this tumor have been reported since 1944. When a 5-year-old female presented with this primary neck mass, questions of diagnosis and treatment were raised. The following case report and discussion will demonstrate the diagnostic methods as well as the course of therapy followed in this type of lesion.
We report a pelvic liposarcoma originating from the left spermatic cord that recurred following inadequate excision. In our case, the tumor was resected without performing orchiectomy previously. The patient was managed by laparoscopic resection, before undergoing radical orchiectomy in the left inguinal region. To our knowledge, no case of laparoscopic resection for the recurrent liposarcoma has been described. In addition, the present case serves to demonstrate that radical orchiectomy with wide excision is needed for paratesticular tumor.
Liposarcomas constitute 15 to 20% of all soft tissue tumors. They are extremely rare in the head and neck and in the oral cavity. A 30-year-old patient was seen for a soft, painless mass in the right palate. Through panoramic radiography it was possible to observe a radiolucent area with sharp margins in the right upper quadrant. The lesion, after an incisional biopsy, was diagnosed as a "myxoid liposarcoma." The patient underwent a wide excision of the lesion with bone laminectomy and he is well at a 4-year follow-up. The differential diagnosis included salivary gland tumors and palatal abscess.
Retroperitoneal liposarcoma is a rare malignancy. We report a case of retroperitoneal myxoid liposarcoma in pregnancy. The patient died of the disease eight months after surgery.
A case of recurrent dedifferentiated liposarcoma simulating malignant fibrous histiocytoma, with complete absence of lipoblastic differentiation, is described. The tumour cells showed strongly positive immunostaining for alpha-1-antichymotrypsin. Electron microscopy revealed a mixture of fibroblasts and histiocytes. Our findings suggest that the dedifferentiated component reflects an altered differentiation pathway of the primitive mesenchymal cells in the original liposarcoma.
The ultrastructure of 10 myxoid liposarcomas from 9 patients is presented. Features closely resembling those of developing fetal adipose tissue were seen in all tumors. THe various cell types identified ranged from undifferentiated cells to typical multivacuolated and univacuolated lipoblasts. In addition, a secretory type mesenchymal cell with distended cisternae of rough endoplasmic reticulum was found in every case as well as intermediate cells with secretory product and lipid accumulation. relative proportions of these types varied from case to case. A prominent feature in all cases was the particular spatial relationship of the abundant capillaries to the various cell types. The undifferentiated cells had perivascular processes with a tendency to envelop the primitive capillaries. These cells progressively enlarged and acquired features of the differentiated cell types as they separated from the lamina. Mitotic activity, when detectable, was prominent in the capillary wall and its vicinity. Lipoblasts resembling those of brown adipose tissue frequently coexisted in the same tumor with others analogous to those of white adipose tissue. Our findings suggest that tumor cells in liposarcoma originate from a dividing population of perivascular mesenchymal cells, in a manner akin to that of developing white adipose tissue.
A myxoid liposarcoma showed macroscopic, histologic, and cytogenetic heterogeneity. In one of three myxoid nodules and in the surrounding lipoma-like tumor tissue, the translocation t(12;16)(q13;p11), known to be specific for myxoid liposarcoma, was found as the sole chromosomal abnormality. In the other two nodules, additional rearrangements involving chromosomes 1, 12, and 16 were found. These aberrations were probably secondary to the primary t(12;16), and are cytogenetic evidence of clonal evolution. The complex chromosome aberrations were present in those tumor parts that had more malignant histology, indicating that the acquisition of secondary chromosomal aberrations parallels the histologic manifestations of tumor progression.
Malignant mesenchymoma is an interesting but very rare tumor in which malignant differentiation has occurred twice or more. We report a case of retroperitoneal malignant mesenchymoma consisting of osteosarcoma, leiomyosarcoma, liposarcoma and fibrosarcoma. Abdominal CT showed a large retroperitoneal mass with two separate and distinct parts, namely an area of prominent calcification and one of clearly enhancing solid components. The mass contained histologically distinct tumorous components with no histologic admixure at the interfaces. The densely calcified nodule corresponded to osteosarcoma, and the noncalcified clearly enhancing nodules to leiomyosarcoma, liposarcoma and fibrosarcoma.