LYMPHOSARCOMA (lymphosarcoma; lat. lympha pure water, moisture + sarcoma; synonym: lymphocytic lymphoma, lymphoblastoma, nekhodzhkinsky lymphocytic lymphoma) — the malignant tumor developing from an adenoid tissue. To a bowl of everything, almost in half of all cases, initially are surprised limf, nodes, went less often. - kish. path, almonds, bones, skin, spleen, soft tissues. L. it is observed at any age, a thicket at males.
Macroscopically L. can have an appearance of separate or multiple tumoral nodes, massive conglomerates and continuous infiltrates.
But gistol, allocate to a structure nodulyarny type L. (see. Lymphoma gigantofollikulyarny ) and diffusion, at Krom tumoral fabric evenly infiltrirut the struck bodies and fabrics, replacing their normal structure.
Diffusion type L., according to the WHO classification (1976), subdivide into six tsitomorfol. options.
1. Lymphocytic L., which fabric is constructed of the tumor cells indistinguishable from normal lymphocytes (fig. 1, 1). Consider that have such structure limf, nodes and other extramedullary tumors at patients hron, a lymphoid leukosis.
2. Limfoplazmotsitarny L., in whose tissue, along with lymphocytes, a large number of plasmocytes meets. L. a similar structure accompany primary macroglobulinemia of Valdenstrem (see. Valdenstrema disease ) and nek-eye to other monoclonal gammapathies. 3. Pro-lymphocytic L. it is constructed by hl. obr. from cells of a transition type between lymphocytes and lymphoblasts (fig. 1, 2), kernels to-rykh can have dredging or look split; nuclear chromatin in them more gentle, than in lymphocytes, but more rough, than in lymphoblasts. Cells pro-lymphocytic L. have strong likeness with leukemic elements of pro-lymphocytic option hron, a lymphoid leukosis and so-called limfosarkomno-cellular leukemia. 4. Lymphoblastoid L. consists of the cells similar blast to cells at an acute lymphoblastoid leukosis (fig. 1, 3), having more basphilic cytoplasm in nek-ry cases. Carry tumors with the so-called twisted kernels of cells to this option, to-rye a thicket occur at children and are located in a mediastinum. 5. Immunoblast L., which cells have the big sizes, plentiful basphilic vacuolated cytoplasm and kernels different, often wrong, forms with gentle network of chromatin and well noticeable kernels (fig. 1, 4). 6. Berkitt's lymphoma, or the African lymphoma (see. Berkitta lymphoma ).
According to nek-ry authors, the majority of L. on immunol, to signs treat derivatives of the V-lymphocytic cellular line. Along with it large number L is described., coming from T - and O-lymphocytes. Characteristic cytochemical, feature of L. detection in tumor cells of CHIC-positive substance in the form of granules is. Distribution of L. lymphogenous and hematogenous; autokhtonny growth by most of authors is denied.
Etiology it is not known; the exception is made by Berkitt's lymphoma which origin is connected with a viral infection.
Clinical picture same type L. it is caused first of all by primary tumor, edges can arise in any body containing adenoid tissue (see). Limf, nodes at the beginning of a disease seldom happen painful and do not cause special disturbance, than and cases of the late address of patients to the doctor speak. Increased limf, nodes have an appearance of separate roundish formations of different density or conglomerates; they can be soldered in continuous tumoral infiltrate (fig. 2), sometimes to germination of skin and an ulceration. Increase retroperitoneal and mesenteric limf, nodes long enough proceeds asymptomatically; increase in the sizes limf, nodes of a mediastinum can cause the expressed syndrome of a prelum; L. almonds — feeling of a foreign body in a throat and difficulties of swallowing; L. a nasal part of a throat — decrease in hearing and difficulty of nasal breath; L. bones — a sharp pain syndrome; at damage of skin, in addition to cosmetic defects, pains at an ulceration are observed; at L. went. - kish. a path — dispepsichesky frustration and a syndrome of disturbance of absorption.
Generalization of L. comes usually early, the local nature of defeat longer others is kept by L. went. - kish. path and nek-ry other localizations. The most frequent zone of innidiation at L. any primary localization are limf. nodes. At primary defeat limf, nodes and a spleen the subsequent distribution of L. occurs preferential on limf, to nodes of the adjacent and remote areas; at primary defeat of an adenoid tissue of a ring of Valdeyer also went. - kish. a path, as a rule, the first metastasises appear in regional limf, nodes. L. skin and soft tissues extend on skin and soft tissues (fig. 3) in the beginning, then nodes and other bodies are involved limf. In metastatic tumoral process, in addition to limf, nodes, the liver, marrow, a spleen, a pleura, almonds, skin, easy, soft tissues, bones and other bodies are involved. The special place in a wedge, a picture L. occupies damage of marrow, during the involvement to-rogo there is a leykemization to development of a picture acute lymphoblastoid, pro-lymphocytic or hron, a lymphoid leukosis according to a form initial L. Damage of marrow occurs for the second time, by seeding from extramedullary tumoral nodes therefore the percent of lymphoid cells in peripheral blood can be higher, than in marrow. Most often the leykemization is observed at primary damage of a spleen, almonds, limf, nodes and can develop in any period of a disease, sometimes very much early; the blast leykemization is usually shown at late stages. Out of the period of a leykemization the composition of blood has no idiosyncrasies. In blood serum at L. sometimes increase in one or, much more rare, two classes of immunoglobulins is defined. It is characteristic that even at extended by L., unlike lymphogranulomatosis (see), most of patients has no symptoms of the general intoxication, ROE does not exceed 30 mm an hour.
Diagnosis put on the basis of results morfol, researches.
Treatment. Single primary L. went. - kish. a path are subject to operational treatment, a cut provides in some cases long remissions. Radiation therapy is shown at limited primary L. almonds, a nasal part of a throat, mediastinal and retroperitoneal limf, nodes, and also at slowly current options L. Preference is given in all other cases to chemotherapy or a combination of chemotherapy with beam treatment. Chemotherapy — the most universal method of treatment of L.: it is shown in all stages of process, is irreplaceable at generalized defeat. From antineoplastic means sarcolysine, Cyclophosphanum, Chlorbutinum, Vincristinum, rubomitsin, Adriablastinum, asparaginase are most effective. At lymphocytic and pro-lymphocytic L. most often apply Chlorbutinum on 10 — 15 mg a day; at lymphoblastoid and immunoblast L. Cyclophosphanum can be appointed. Frequency of remissions at treatment of L. one some himiopreparat, including Cyclophosphanum, according to many authors, does not exceed 50 — 60%. Therefore treatment is carried out by a complex of drugs that gives the best effect. So, e.g., Cyclophosphanum with Vincristinum (Oncovinum) and Prednisolonum provide approach of remission in 70 — 80% of cases. Apply also other complexes. For prevention of a recurrence the maintenance therapy is carried out, appoint courses of a reinduktion of remission.
The forecast depends on type and option of a structure of L.: at nodulyarny L. it is more favorable, than at diffusion; lymphocytic and pro-lymphocytic L. L proceed dobrokachestvenny, than lymphoblastoid and immunoblast.
Bibliography: Non-Hodgkin’s lymphomata, Brit. J. Cancer., v. 31, suppl. 2, 1975.
G. B. Kruglova.