Primary brain lymphomas in patients with a prior or concomitant malignancy.

The increased incidence of second malignancies among cancer survivors is well documented. Thus, differential diagnosis between metastatic spread from a prior malignancy and the occurrence of a new neoplasm should be considered. This is particularly difficult for brain lesions due to their poor prognosis that often discourages diagnostic work-up. In some cases diagnosis of a second primary neoplasm, such as primary central nervous system lymphomas (PCNSL), could change the therapeutic management and the prognosis. About 8% of PCNSL occurs as a second malignancy. Homogeneous and intense tomographic enhancement, deep location of lesions and dramatic response to corticosteroids are suggestive for PCNSL and should be carefully considered before the start of treatment for cerebral lesions. Prognosis and standard management of brain metastases and PCNSL are almost completely different. In addition, while treatment of brain metastases often has a palliative purpose the goal in PCNSL treatment is the cure. Four patients with PCNSL as a second malignancy are reported and literature is reviewed. Diagnosis of PCNSL changes the strategy of treatment which could have a critical therapeutic and prognostic impact.

Large-cell variants of mantle cell lymphoma: cytologic characteristics and p53 anomalies may predict poor outcome.

Large-cell variants are uncommon in mantle cell lymphoma (MCL). Here we describe the pathologic and clinical findings in five patients with large-cell lymphoma related to MCL (L-MCL), and compare them to a group of classic small-cell MCL (s-MCL) cases. Histologically, the MC origin of the large cells was evinced by their association with a small mantle cell component in the same tissue, or their distribution in a classic mantle zone pattern, or their development in a patient with previous s-MCL. The large cells were either pleomorphic mantle cells (case 1) or transformed blast-like cells (case 2-5). The median nuclear diameter, median nuclear area and proliferation index of L-MCLs and s-MCLs, were statistically different. Immunophenotypic characterization of four specimens of L-MCL and 10 of s-MCLs with a large panel of antibodies showed the classic findings of MCL, i.e. the IgM+ D+/-, CD5+, CD10-, CD23- phenotype in all cases except two (one CD5- and one CD23+), and the association with a loose follicular dendritic cell network. Two of four L-MCLs and 5/10 s-MCLs demonstrated rearrangements of the bcl-1 gene by Southern blot or by polymerase chain reaction (PCR); 2/4 L-MCLs and 1/9 s-MCLs had p53 mutations on single-strand conformation polymorphism analysis; none of the 14 specimens showed rearrangement of bcl-2 by PCR or bcl-6 and c-myc by Southern blot. All patients with 'transformed' histology (versus 37% of all others) died of lymphoma; their survival (15-18 months; median 17) was much shorter than that of all the others (28-117+ months; median 43) (P=0.0035). All three patients with p53 anomalies, two of whom had tumours with transformed histology, died of their disease in a short time (15, 18 and 28 months). In contrast, the presence of bcl-1 rearrangements did not have prognostic implications. This study documents the existence of large-cell variants of MCL and the poor prognosis associated with the 'transformed' cytologic type and/or p53 abnormalities.

Importance of complete staging in non-Hodgkin's lymphoma presenting as a cerebral mass lesion.

BACKGROUND: The high incidence of local failure and the lack of evidence of systemic dissemination of primary central nervous system lymphoma (PCNSL) has led some authors to conclude that complete staging with extensive tests is not necessary in patients with a lymphomatous cerebral mass who present with a focal neural syndrome. Moreover, De Angelis emphasizes that there is no report in the literature of systemic lymphoma presenting as a cerebral mass lesion, whereas others conclude that an extensive systemic evaluation is unnecessary or that the staging should be limited to physical examination, routine blood studies, and a chest radiography. METHODS: In 16 PCNSL patients observed between 1982 and 1992, we found two patients showing systemic involvement of lymphoma after staging workup with chest X-ray, total body computerized tomographic (CT) scan, cerebrospinal fluid cytology examination, and bone marrow biopsy. In one patient, a CT abdominal scan showed retroperitoneal and pelvic lymphonodal involvement, whereas in the second patient, bone marrow biopsy was positive. These systemic onsets were found no later than 2 months after the diagnosis of central nervous system involvement, which is sufficient time to conclude that these locations were present at the diagnosis of the cerebral mass. RESULTS: In the present study, complete staging demonstrated widespread dissemination (lymph nodes or bone marrow) in two patients with initial neurologic symptoms and a cerebral mass lesion. CONCLUSIONS: In our experience, complete staging allowed detection of systemic lymphoma in two patients initially considered to have PCNSL.

Bone marrow biopsy in 50 AIDS patients: a diagnostic approach.

Fifty bone marrow biopsies (BMB) and aspirates (BMA) of AIDS patients were examined to identify possible morphologic signs of HIV infection. The following parameters were evaluated: 1) cellularity; 2) myelo-erythroid ratio (M:E); 3) morphology of haemopoietic lineages; 4)plasmacell amount; 5) lymphocytes amount; 6)reticulin content. Hypercellularity, increase in number and morphological alterations of megakaryocytes, raised reticulin content, mild plasmacytosis and frequent presence of reactive lymphoid aggregates are features suggestive, thought not diagnostic, for HIV infection on BMB. It results that bone marrow morphologic examination in HIV-positive patients plays a distinctive role in ruling out the presence of opportunistic infections or associated neoplasias (haematologic or not) and in contributing to clarify the significance of a peripheral pancytopenia.

Splenic lymphoma: unusual case with exclusive red pulp involvement.

A case of Primary Malignant Lymphoma of the Spleen (PMLS) with exclusive red pulp involvement is described and discussed. Although the unusual topographic presentation the authors emphasize the physiologic arrangement of lymphoid cell in splenic red pulp cords that can give origin to the neoplasia. They moreover discuss problems of differential diagnosis with Malignant Histiocytosis (MH), Hairy Cell Leukemia (HCL) and Myeloid Process, both by morphology and immunohistochemistry.

Hairy cell leukemia without splenomegaly nor myelofibrosis in a patient with gastric adenocarcinoma: early phase of the disease or a variant?

The first case of patient affected both by gastric carcinoma and hairy cell leukemia (HCL) is reported. From a clinical standpoint, this 54-year old man presented with striking leukopenia without splenomegaly. From a morphological point of view, the infiltration by leukemic cells occurred in the hypoplasic areas of the bone marrow biopsy (BMB) without increase in reticulin fibers. From these observations the authors deduce that: 1) BMB in many cases is the only morphologic tool for diagnosis and prognosis of HCL; 2) probably the "hypoplasic" variant of HCL will become more frequent, because of the increasing indication to BMB in course of pancytopenia, even in absence of splenomegaly; 3) our case is probably related more to an early phase of the disease than to a distinct variant. In addition, we propose that the co-existence of an aggressive solid tumor and HCL could be related to the immunosuppressive action by hairy cells.

[Monoclonal gammopathies: differential diagnosis with bone marrow biopsy]. / Le gammopatie monoclonali: diagnostica differenziale su biopsia osteomidollare.

Aim of the present study is to examine the morphological aspects of monoclonal gammopathies (GM) detectable by bone marrow biopsy. With reference to Multiple Myeloma (MM), the more important disease of this group from a clinical standpoint, other conditions will be later evaluated. Particular attention will be given to Monoclonal Gammopathy of Undetermined Significance (MGUS) that still today is characterized by wide shadows concerning both ethiology and evolution significance.