RETICULOSARCOMA OF THE BONE PRIMARY

From Big Medical Encyclopedia

RETICULOSARCOMA OF THE BONE PRIMARY (Latin reticulum grid + sarcoma; synonym: monocytome of a bone, retikulokletochny sarcoma of a bone) — solitary malignant new growth of a bone of a reticular and cellular origin. It is localized usually in long tubular bones, arises in flat bones or other departments of a skeleton less often. It is for the first time allocated as a nosological form in 1939 by Parker and Jackson (F. Parker, N. to Jackson).

River to. the item makes apprx. 3% of all primary tumors of a skeleton. More often males at the age of 15 — 30 years get sick.

Etiology it is unknown. Cases of emergence of R. are described to. the item on the basis of Pedzhet's disease (see. Pedzheta disease ).

Originally the tumor is always located vnutrikostno with the subsequent partial or final fracture of compact substance. The tumor is presented by the center of soft gray-pink tissue which does not have a clear boundary. Rather often at R. to. the item in a zone of the center of defeat is observed patol. a change with signs of secondary reparative and reactive bone formation.

Fig. 1. Microdrugs of primary reticulosarcoma of a bone: and — all field of vision is busy with tumor cells with the expressed atipizm; coloring hematoxylin-eosine, x 250; — the network of the argentofilny fibers braiding tumor cells is visible; impregnation by silver, x 250.

Microscopically the basic structural element are tumor cells of lymphoid and reticular type with more or less expressed signs of polymorphism and atypia with oval, fabiform or horseshoe kernels and rather accurately konturirovanny cytoplasm (fig. 1, a). R.'s cells to. the item by the sizes is slightly larger than cells of sarcoma of Ewing (see. Ewing tumor ). Sometimes - cells contain two or three kernels, usually with chromatin in the form of rough irregular shape glybok. Figures of a mitosis are often observed.

Unlike Ewing's tumor in R. to. the item almost constantly is possible to reveal rich network of argentofilny fibers, to-rye sometimes braid almost each cell (fig. 1, b). In cytoplasm of cells of R. to. the item does not contain a glycogen. In peripheral departments of a tumor signs of a kollagenoobrazovaniye are often observed sometimes up to formation of small fibrous fields. In the same sites of a tumor it is possible to see also the centers of reactive bone formation.

Clinically the disease differs in the gradual beginning. The first symptoms are pains in a zone of the center of defeat with irradiation along nervous trunks as neuritis or radicular pains. In the beginning pains intermittent, sometimes arise only at an exercise stress and only in several months gain constant character. In nek-ry cases of pain are localized in the next to the center of defeat a joint. A part of patients (apprx. 30 — 40%) addresses for medical aid already in the presence patol. change. Therefore inflammatory and reactive changes quite often complicate diagnosis.

River to. items in 20 — 25% of cases metastasize in bone system, most often in flat bones of a skull, a shovel, pelvic bones and edges.

The diagnosis is established by means of complex clinical, rentgenol., radio isotope and pestilence foul. researches.

Fig. 2. Roentgenograms of a femur (a) and bones of a shin (—) at primary reticulosarcoma of a bone (a direct projection): and — the small widespread centers of destruction in an upper part of a diaphysis of a femur; — destruction and stratification of compact substance throughout all diaphysis of a fibular bone with considerable endosteal reaction; in — the cystic cellular centers of destruction in a meta-diaphysis of a tibial bone (are specified by shooters); — the center of destruction in a distal metadiaphysis of a fibular bone (1) with periosteal growths (2).

Rentgenol. the picture is diverse and depends on a growth phase and localization of a tumor. In the beginning there is a site of destruction of fine, porous, spotty grained structure, to-ry is located in spongy substance (fig. 2, a). Further lytic process accrues, the centers of destruction of a rounded or oval shape with indistinct outlines form. Discrepancy between a little expressed a wedge, symptomatology and considerable rentgenol is often observed. changes. It is caused by the infiltrative growth of a tumor, formation of its extra bone component, to-ry does not contain limy inclusions. Lamellar destruction of compact substance can come to light (fig. 2, b). Destructive process is sometimes characterized by existence of large tumoral nodes with dense edges and various degree the «swelling» of a bone reminding a bone cyst, and in the presence of a large number of crossbeams — an osteoblastoclastoma (fig. 2, in, d).

Endosteal reaction can be in the form of a sclerous border on border of the large center of destruction or focal consolidation at melkoochagovy destruction. Very seldom sharp consolidation of a bone meets, at Krom the marrowy channel is not differentiated.

Destructive changes are followed by various periosteal reaction — a layered, lacy, fringed and spikuloob-different periostosis. The linear periostitis is the early symptom which is coming to light before spread of a tumor to soft tissues.

For R.'s identification to. the item and its metastasises the importance belongs to a radio isotope research. At the same time it does not give ideas of character patol. process, however allows to specify the extent of a tumor in the affected bone, and also to establish existence or lack of metastasises in other departments of a skeleton. By means of a radio isotope research metastasises come to light earlier, than at a X-ray analysis. The most accurate visualization of changes is possible during the use 99m Tc-pyrophosphate, to-ry gives a high level of accumulation in area of a tumor and its metastasises. In doubtful cases it is reasonable to conduct a research in the beginning with 85 Sr, having smaller sensitivity (poorly positive level of accumulation in the affected bone), but higher specificity in comparison with 99m Tc-pyrophosphate. Results of such research can matter in differential diagnosis with Ewing's sarcoma, a lymphosarcoma, damage of marrow at a multiple myeloma, and also other malignant tumors of bones and metastasises in a bone, at to-rykh the level of accumulation 85 Sr is higher.

At treatment River to. the item preference is given to radiation therapy. An optimum focal total dose at the same time consider 5000 — 6000 I am glad (50 — 60 Gr). For the prevention of generalization of a tumor recommend the combined treatment using along with radiation therapy (for primary center) to system chemotherapy drugs like sarcolysine or Cyclophosphanum. An operative measure is recommended only in cases of insufficient effect of conservative treatment.

Forecast adverse, 5-years-nyaya survival makes, according to different researchers, from 28 TO 75%.

See also Bone (tumors) .



Bibliography: Vinogradova T. P. Tumors of bones, page 198, M., 1973; Histologic classification of bone tumors, sost. F. Shayovich, etc., the lane with English, page 40, M., WHO, 1974; M e h e in D. M., etc. Comparative assessment of the separate and combined use of strontium-85 and technetium-99m of a pyrophosphate for radiodiagnosis of tumors of a skeleton, Ortop. and travmat., No. 12, page 11, 1980; Pathoanatomical diagnosis of tumors of the person, under the editorship of N. A. Krayevsky, etc., page 413, M., 1982; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, book 2, page 438, M., 1964; Lichtenstein L. Bone tumors, p. 329, St Louis, 1977; Parker F. Jackson H. Primary reticulum cell sarcoma of bone, Surg. Gynec. Obstet., v. 68, p. 45, 1939; Van den Bout A. H. Malignant lymphoma (reticulum cell sarcoma) of bone, S. Afr. med. J., v. 57, p. 193, 1980; W i n k e 1 U. Das primare Reti-kulumzellsarkom des Knochens, Hamburg, 1971.


BB. H. Nightingales; AA. 3. Novikova

(rents.).

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