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Malignant Lesions Besides primary retroperitoneal sarcomas, other systemic cancers may manifest with retroperitoneal masses and need consideration in the differential diagnosis. Retroperitoneal lymphoma may manifest with retroperitoneal diffuse lymph node enlargement. Occasionally the involved lymph nodes will coalesce to form irregular masses that are indistinguishable from other primary RPTs. Lymphoma may even mimic retroperitoneal fibrosis with a homogenous dense midline mass that resembles fibrosis.

Metastatic germ cell tumors may lead to retroperitoneal lymphadenopathy that may be bulky. Typically such masses deflect the ureters laterally, but may occur in between or anterior to the great vessels. The diagnosis of germ cell tumor can be established Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum by finding a testicular mass and elevated relevant serum markers. Lymphoma may be associated with splenomegaly, elevated lactate dehydrogenase, and other symptoms.

Primary mesenchymal tumors can be classified according to the mesenchymal tissue component of origin. Table 59-1 depicts the classification of benign and malignant mesenchymal tumors according to the mesenchymal tissue of origin.

Liposarcoma is by far the most common type of retroperitoneal sarcoma. Several classifications of these have been proposed. Enzinger and Winslow (1962) modified a previous classification by Stout and proposed five categories: (1) myxoid, (2) welldifferentiated, (3) round cell, (4) de-differentiated, and (5) pleomorphic. The first Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum are considered low-grade and the last three high-grade sarcomas. Myxoid liposarcomas are composed of primitive lipoblasts hexal torasemide do not have the typical fat-laden cytoplasm but rather resemble primitive mesenchymal cells.

Abundant capillary network and myxoid matrix are other typical components. The histologic appearance of well-differentiated liposarcoma closely resembles that of a benign lipoma, and the distinction between the two by imaging and even under the microscope is a challenge.

In fact, many well-differentiated liposarcomas are misdiagnosed as deeply seated lipomas. Although well-differentiated liposarcomas seldom metastasize, local recurrence is common Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum long-term prognosis is influenced by the morbidity caused by such recurrences involving other organs and the morbidity of the necessary surgeries.

Round cell liposarcoma is composed of small round cells uniform in size and closely packed together. There is no specific pattern of Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum arrangement and intracellular lipid content is scarce. De-differentiated liposarcoma is characterized by the coexistence of well-differentiated and poorly differentiated areas within the same tumor.

Occasionally, at the time of local recurrence other phenotypes may be present, including malignant fibrous histiocytoma, rhabdomyosarcoma, or leiomyosarcoma. Characteristic features of pleomorphic liposarcoma include a disorderly growth pattern with cellular pleomorphism, giant cells, and anaplastic pyknotic nuclei. Because this anaplastic tumor resembles other undifferentiated sarcomas, some lipoblastic presence must be documented to confirm this diagnosis.

All lipocytes and lipoblasts stain positive with the immunostaining agent S-100, rendering this a useful tool in establishing this diagnosis.

Ring chromosome 12 is typical of well-differentiated liposarcomas but also has been demonstrated in benign lipomas (Dal Cin et al, 1993). Malignant fibrous histiocytoma has been the subject of controversy from both an ontogenetic and diagnostic standpoint. Although its name implies that histiocytes are the building block and cell of origin, truly this is a fibroblast neoplasm.

Microscopic findings include round histiocyte-like cells, spindle-shaped fibroblasts, foamy cells, maslow hierarchy of needs cells, and lymphocytes. Several subtypes have been reported; whereas the myxoid subtype is associated with a somewhat more favorable prognosis, the other subtypes are aggressive and show a high tendency to metastasize.

In addition, some studies have shown an association between the presence of lymphoproliferative disorders, including leukemia, and both Hodgkin and non-Hodgkin lymphoma, and the development of malignant fibrous histiocytoma.

The cause of this apparent relationship between malignant fibrous histiocytoma and hematologic malignancies remains unclear. Retroperitoneal leiomyosarcomas usually occur in women in their 7th decade.

The tumors mediline very large size and include cystic degeneration and necrosis. Microscopic findings include spindleshaped cells with abundant cytoplasm and cigar-shaped nuclei. As is the case with well-differentiated liposarcoma, distinction of a leiomyoma from a leiomyosarcoma is difficult even under rigorous microscopic review.

Parameters suggestive of malignancy include 1406 PART X Neoplasms of the Upper Urinary Tract tumor size, pleomorphism, cellularity, necrosis, atypia, and mitosis.

Of these, mitosis is the most highly predictive feature and in RPTs 1 mitosis per 10 high-power fields (HPFs) is characteristic of malignancy, whereas more mitotic figures are tolerated in smooth Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum tumors in other anatomic locations.

Tumor grade is an important prognostic factor because high-grade Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum are associated with a less favorable outcome. Of these, the mitotic index (number of mitoses per 10 HPFs) and necrosis are independent prognostic factors whose impact is variable depending on tumor type (El-Jabbour et al, 1990; Hashimoto et al, 1992; Catton et al, 1994).

In addition, molecular markers, including ploidy, chromosomal aberrations, proliferative index, and tumor promoter and suppressor gene mutations have been shown mp43 influence outcome. STAGING OF RETROPERITONEAL SARCOMAS CLINICAL PRESENTATION AND WORKUP Hematogenous spread is the principal route of metastasis for sarcomas, and the lungs are the most common metastatic site for such tumors, followed by the liver.

Because cross-sectional imaging is necessary as the initial diagnostic workup, liver involvement will be picked up in the initial imaging procedure. Chest computed tomography (CT) is required for all retroperitoneal fragile syndrome x to detect pulmonary metastasis. Other sites such as bones and brain are infrequently involved, and routine imaging of these sites using brain MRI and bone scintigraphy is not required in the absence of relevant symptoms.

The exceptions may be lymphangiosarcoma, osteogenic sarcoma, thin Ewing sarcomaall of which also may involve the skeleton, and bone scans should be obtained in such patients. The Naloxone Hydrochloride Nasal Spray (Narcan Nasal)- Multum, node, metastasis (TNM) staging system has been used to define the local and systemic extent of tumors as follows: Before histologic diagnosis of an RPT is available, its presence is typically heralded by imaging findings either as part of a workup for a suggestive clinical presentation or as an incidental finding on an imaging procedure undertaken for other unrelated reasons.



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