LEUKEMIC RETICULUM-CELL SARCOMA (RETICULUM-CELL SARCOMA TERMINATING IN ACUTE LEUKEMIA). REPORT OF TWO CASES AND REVIEW OF THE LITERATURE.
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Reticulum cell sarcoma involving the vitreous and the brainstem occurred in a 45-year-old man. He initially was seen with diplopia from a partial left-sided third cranial nerve palsy, which is rare. Later, a typical uveitis developed in the right eye. An initial diagnosis of brainstem glioma, based primarily on the computed tomographic scan findings and clinical history, was ultimately proved erroneous when the correct diagnosis was shown by the results of a cytologic examination of vitreous aspirate. Excellent visual response to a moderately high oral dose of steroids occurred, which has not been usual in other reported cases. Definitive cobalt (gamma) radiation therapy (6,000 rad to the brainstem and 4,000 rad to the vitreous) has produced a one-year remission at this time.
Neoplasms of reticular dendritic origin are extremely rare and include the follicular dendritic cell sarcoma (FDCS) and the interdigitating reticulum (or dendritic) cell sarcoma (IDCS). In this article, we review the literature pertaining to the two diseases and describe clinical observations and salient pathologic features, including information provided by authors of FDCS and IDDCS reports. We performed a computerized database search for published articles regarding FDCS and IDDCS. The articles were evaluated critically by the authors. Simple descriptive statistics were used to analyze the data. There are 51 cases of FDCS and 21 cases of IDDCS that are well documented in the literature. The pathologic diagnosis of FDCS and IDDCS is often challenging and requires morphologic, immunophenotypic, cytochemical, and electron-microscopic analysis. Patients with FDCS usually present with cervical or axillary lymphadenopathy, but extranodal disease has been described. In at least some patients, preexisting Castleman's disease has been recognized. Resected localized disease may be prevented from recurrence by consolidative radiotherapy. Chemotherapy regimens have shown nondurable antitumor activity in FDCS. Patients with IDDCS usually present with lymphadenopathy. The clinical course of IDDCS has been variable, but it seems to be more aggressive than that of FDCS. Variable degrees of remission may be achieved with chemotherapy. FDCS and IDDCS are rare neoplasms that may pose difficulty in pathologic diagnosis. IDDCS seems to display a more aggressive behavior than FDCS. Patients with IDDCS and FDCS can eventually die of disease progression. The role of chemotherapy and radiotherapy is not clearly defined.
A reticulum cell sarcoma (RCS) which initially metastasizes selectively to the spleen in C3H/HeN mice has been studied. Previous reports indicated that removal of the spleen results in widespread metastases of this tumor. The current experiments utilized a parabiotic system to determine the effect of additional splenic tissue on the gross and microscopic metastatic pattern of this RCS. The results were that the addition of a second spleen in the parabiotic group was followed by a significant decrease in visceral metastases compared with the splenectomized group. Parabiotic animals with splenectomized partners and sham-operated animals did not significantly differ from control (single) animals, with all three of these groups showing visceral metastatic spread intermediate between the parabiotic and splenectomized groups. The protective effect of additional spleen against visceral metastases was found not to be immunologic, since immunosuppression of mice by irradiation produced a metastatic pattern similar to that of control animals.
The reticulum cell sarcomas (RCS) of SJL/J mice are of particular interest since they readily induce the proliferation of syngeneic T-lymphocytes. Previous cellular studies examined the antigens on the RCS which stimulated this response and suggested that the tumor expressed allogeneic I-region-associated (Ia) antigens normally associated with the E alpha:E beta molecular complex (S. M. Wilbur and B. J. Bonavida, Exp. Med., 153: 501-513, 1981). These particular Ia glycoproteins are not expressed on normal SJL/J cells due to a defect in the E alpha polypeptide synthetic pathway. However, the E beta subunit is synthesized normally by these animals but remains intracellular. The SJL/J-derived RCS may circumvent this defect in E alpha subunit biosynthesis. The aberrant synthesis of this polypeptide is thought to allow membrane presentation of an intact pseudoallogeneic Ia glycoprotein which utilized the normally dormant E beta s polypeptide. In the present study, two monoclonal antibodies directed against the Ia.7 specificity of the E alpha chain (13/18, 14-4-4S) were used to examine more directly the expression of this polypeptide on the tumor. Surprisingly, neither antibody was effective against the RCS in a direct complement-mediated cytolysis assay. Nevertheless, the tumor was found to specifically adsorb lytic activity of both the monoclonal antibodies. In addition, both a cold-cell competition assay and indirect immunofluorescence corroborated the data and indicated that the RCS does express detectable levels of the Ia.7 antigen. Normal spleen cells and lipopolysaccharide B-derived blasts from SJL/J mice were found in all experiments to be devoid of any specific reactivity with these monoclonal antibodies. In addition, continued in vivo passage of transplantable RCS was found to cause down-modulation of the Ia.7 specificities on these tumors. Newer RCS transplantable lines, however, expressed demonstrable levels of this alloantigen in both cellular and serological assays. The observed down-modulation could explain the difficulties encountered in defining this specificity on long-term transplantable RCS. In conclusion, the present serological study corroborates the early cell-binding data. An Ia.7 antigen is shown to be expressed on the RCS, yet this specificity could not be detected on normal SJL/J cells.
Spontaneous reticulum cell sarcoma in SJL/J mice has been proposed as an animal tumor model for Hodgkin's lymphoma. The relationship of tumor progression and immune function is not clear, however, and has prompted a systematic evaluation of SJL/J immune competence. It was found that the ability to generate cell-mediated immunity and antibody response to allografts was not impaired in 2- to 12-month-old mice, regardless of their tumor status. All animals were capable of generating in vivo cytotoxic effector T-cells and both IgG and IgM classes of cytotoxic antibody to a tumor allograft. In addition to being able to respond, older mice showed an unexpected hyperresponsiveness to alloantigens, which suggested that escape from feedback control might be a characteristic of SJL/J mice. Loss of immune regulation was further indicated by the failure to induce tolerance to human gamma-globulin in mice 4 months and older, while 3-week-old SJL/J mice could be rendered unresponsive. Coincident with this apparent loss of regulation, circulating antibodies to synthetic double-stranded RNA, polyinosinis - polycytidylic acid, were first detected in unimmunized mice at 4 months of age, and titers remained elevated regardless of tumor status. It is suggested that tumor development as well as autoimmunity may result from an effective amplification of the immune response.
Electrophoretic mobility of cells from 21 primary mouse leukemias was investigated and compared with that of various normal mouse reference cells. Six thymic lymphomas, nine reticulum cell sarcomas, five myeloid leukemias and one stem-cell leukemia were examined. The mean AEM of cells from the group of primary thymic lymphomas (1.13 microns s-1 V-1 cm) was similar to that of reticulum cell sarcomas (1.15 microns s-1 V-1 cm) and significantly lower than that of the myeloid leukemias (1.20 microns s-1 V-1 cm). Since the examined cell suspensions were prepared from leukemic lymph nodes, the analysis was performed to investigate the possible admixture of normal LNC in the cell populations. Electrophoretograms of normal and leukemic lymph nodes were compared and the AEM of cells from leukemic lymph nodes was considered separately for two cell populations, one corresponding in size to normal LNC (6-9 microns in diameter) and the other to leukemic blast cells (10-16 microns in diameter). In the 6-9 microns cell subpopulations from leukemic lymph nodes, electrophoretically slow cells corresponding to B LNC were almost totally depleted (1%); also electrophoretically fast cells with the mean AEM of T LNC were fewer (45-57%) than in normal LNC populations. The leukemic cells 10-16 microns in diameter displayed mean AEM of 1.09 (thymic lymphomas), 1.12 (reticulum cell sarcomas) and 1.21 (myeloid leukemia) micron s-1 V-1 cm. The mean AEM of blast cells from thymic lymphomas and reticulum cell sarcomas was not significantly different; it was similar to that of blast cells prepared from normal mouse thymus (1.09 microns s-1 V-1 cm) on a density gradient. In contrast, the 10-16 microns cell populations from lymph nodes of mice with myeloid leukemias were significantly faster than blast cells from thymic lymphomas and reticulum cell sarcomas.
In a 43-year-old man who for 20 years was known to have dermatitis herpetiformis there co-existed, since 1974, a "dermatogenic enteropathy" with gastro-intestional symptoms. Several small-intestine biopsies demonstrated severe partial to subtotal villous atrophy with crypt hyperplasia, as seen in gluten-sensitive enteropathy. At post-mortem there was an immunoblastic sarcoma - so-called reticulum-cell sarcoma - in the upper jejunum and abdominal lymph-nodes, which was presumably the immediate complication of the sprue-like lesions of the small intestines.
The results of studies on the reticulum cell sarcoma (RCS) tumors of SJL/J mice presented here, indicate that spontaneous tumors, which arise in older mice, also possess the capacity to induce the vigorous proliferative response in syngenetic T lymphocytes that are characteristic of the transplantable RCS lines. Analysis of cell surface antigens revealed the presence of Ia determinats on gradient-purified transplantable RCS tumor cells; however, these cells did not express Thy 1.2, nIg, or, any of the viral proteins that were tested for by specific antisera. Pretreatment of RCS cells with anti-Ia sera and complement-deleted cells that were stimulatory for syngenetic T lymphocytes, and addition of anti-Ia sera directly to cultures blocked the proliferative response at the stimulator (RCS) cell level. Lymph node cells from H-2(8) strains other than SJL/J, including A.SW and B10.S also gave proliferative responses to RCS cells, although lower in magnitude. A requirement on the part of responding cells for identity with RCS cells at the Ir region was indicated by the finding that A.TH but not A.TL lymph node cells responded to RCS. It is concluded that RCS cells stimulate Ir-region identical T cells (without evidence of presensitization) through a modification in the expression of Ia antigens on the surface of the tumor cells.
Primary cerebral reticulum cell sarcoma appeared as unexplained posterior uveitis. A retrospective review of 19 cases of cerebral reticulum cell sarcoma seen at Massachusetts General Hospital has not confirmed the previously described high incidence of ocular involvement in patients with this tumor. The presence of posterior uveitis remote from a definite cerebral mass should suggest the possible diagnosis of primary cerebral reticulum cell sarcoma.
Production of interleukin 2 (IL-2) in mixed lymphocyte cultures of SJL/J lymph node (LN) and gamma-irradiated, syngeneic lymphoma cells of transplantable reticulum cell sarcoma (gamma-RCS) was abolished by pactamycin pretreatment of gamma-RCS. LN cells needed for this interaction were Lyt-2 T-cells, not adherent to Sephadex G-10. The effect of separation of T-cells and gamma-RCS after the first 24 hours of culture suggested that both components contributed to the IL-2 production. Exogenous IL-2, but not interleukin 1 (IL-1), restored the ability of pactamycin-treated gamma-RCS to induce syngeneic T-cell proliferation. The inability of T-cells from "nonresponder" F1 hybrids of SJL to proliferate to gamma-RCS was not corrected by addition of IL-1 or IL-2. Interferon (IFN) production also required the presence of untreated gamma-RCS and LN cells, but it was dependent on a Sephadex G-10 and plastic adherent cell in LN. IFN-free supernatant of LN cells plus gamma-RCS induced IFN production in fresh normal lymphoid cells, suggesting a possible indirect induction of this lymphokine. In addition, unirradiated RCS cells (la+ cells) produced immune IFN over a period of 3 weeks in vitro, after which the cells lost their viability. Prolonged IL-2 production was not observed in these cultures. The possible biological importance of the lymphokine production during the exaggerated syngeneic mixed lymphocyte reactions induced by RCS cells is discussed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Using the reticulum cell sarcoma (RCS) of the SJL/J mouse as a model for Hodgkin's disease, the effects of tumor growth and splenectomy on the T-cell and B-cell populations were evaluated. Although splenectomy improved survival and reduced gross observable tumor growth in secondary tumor sites, no effect on T-cell and B-cell populations was detected. We conclude that tumor growth appears to be undetected by the immune system; previously reported immunosuppression in RCS-bearing mice must be due to functional changes and not cell population changes; and splenectomy contributes to survival and suppression of tumor cell growth in secondary sites.
The M5076 reticulum cell sarcoma is a murine tumour of potential value in experimental chemotherapy. Experiments were conducted to ascertain the growth characteristics and chemosensitivity of this neoplasm in the BDF1 mouse. The intramuscular tumour proved to be responsive to the alkylating agents, nitrosoureas, procarbazine, DTIC and treosulphan, yet insensitive to the antimetabolites and only weakly responsive to adriamycin. Analogues of the antitumour agents hexamethylmelamine and N-methylformamide were tested against this neoplasm. The patterns of activity determined for these analogues against this tumour were identical to those previously reported against other model systems.
Murine reticulum cell sarcoma (M5076) was subcutaneously implanted into BDF1 mice and then the antitumor activity of seven micelle-forming type platinum complexes was tested. The antitumor activity of bis(hyodeoxycholato)-trans-(+/-)-1,2-cyclohexanediammineplatinu m(II)(t-DACHP (hyo)2) was highest (95% inhibition of growth), and it was dose dependent with a large therapeutic index. This was followed by bis-(chenodeoxycholato)-trans(+/-)(cis)-1, 2-cyclohexanediammineplatinum(II)(t(c)-DACHP-(cheno)2) (49% inhibition) and bis(ursodeoxy-cholato)-trans(+/-)-1,2-cyclohexanediammine platinum (II) (t-DACHP(urso)2) (48% inhibition). t-DACHP(hyo)2 and t-DACHP(urso)2 inhibited sarcoma 180 growth (63% and 33%, respectively). The organ distribution of the complex with the highest antitumor activity was compared with that of a complex with negligible antitumor activity. The total Pt levels were significantly higher in tumor tissue from mice given the more active complex than in tumor tissue from mice given the less active complex. Pt levels in the kidney and the spleen showed a similar pattern, but the lung tissue Pt levels were significantly higher in mice given the less active complex.
A case of reticulum cell sarcoma associated with involvement of the central nervous system was presented. This seems to be an atypical case of reticulum cell sarcoma for the following reasons. 1. Hepatosplenomegaly and enlarged lymph nodes were not observed through the entire clinical course. The main abnormalities were psycho-neurological. 2. These cells did not form a tumour mass, but infiltrated diffucely into the central nervous system and extraneural sites, and the primary focus could not be determined. Four cases of reticulum cell sarcoma in Japan, in which the initial symptoms were of neurological involvement, are reviewed.
Although rare, ocular reticulum cell sarcoma presents a recognisable clinical pattern, as confirmed by 3 new cases. Typical patients, in their sixth and seventh decades, initially complain of gradual visual loss. Examination reveals 'uveitis' with prominent vitreous debris and/or chorioretinal infiltrative lesions. Topical steroid and mydriatic therapy is ineffective. Reticulum cell sarcoma in the central nervous or other systems may precede or accompany the ophthalmic presentation. In an increasing number of cases tissue diagnosis and effective therapy have followed vitreous aspiration.