Surgical treatment of primary tumor in the right heart]

From April 1986 to March 1990, 5 patients with primary tumor of the right heart were treated surgically at our hospital. Two of them had right atrial myxomas, and one had malignant lymphoma of the right atrium. The remaining 2 patients had tumors of the right ventricle (fibromyxoma 1 patient and leiomyosarcoma 1). The diagnosis was confirmed by 2-D echocardiography in all patients. Three patients with cardiogenic shock were operated on at emergency clinic. There were no operative deaths. Two patients with primary malignant tumors underwent incomplete resection with poor results. Three patients with benign tumors who had the tumors totally removed showed good results after operation.     

Anaplastic large cell lymphoma associated with breast implants: a report of 13 cases

We report 13 cases of anaplastic large cell lymphoma (ALCL) associated with breast implants. Patient age ranged from 39 to 68 years, and the interval from implant to ALCL was 4 to 29 years. All tumors were composed of large, pleomorphic cells that were CD30 and ALK1, and all 7 cases assessed had monoclonal T-cell receptor γ-chain rearrangements. Two patient subgroups were identified. Ten patients presented with effusion surrounded by fibrous capsule without a grossly identifiable tumor mass. Nine patients had stage I and 1 had stage II disease. Eight patients underwent implant removal and capsulectomy. Four patients received chemotherapy and 4 radiation therapy. All patients were alive without disease at last follow-up. A second subgroup of 3 patients had effusion and a distinct mass adjacent to the implant. One patient had stage I and 2 stage II disease. One patient had a 3-year history of lymphomatoid papulosis, and 1 patient had a 1-year history of CD30 T-cell lymphoma adjacent to the breast before the diagnosis of ALCL associated with breast implant. Two patients received chemotherapy and 1 radiation therapy. Two patients died 2 and 12 years after diagnosis, respectively. We conclude that the clinical behavior of ALCL associated with breast implants is heterogeneous. Patients who present with effusion without a distinct mass have an indolent disease course, similar to CD30 lymphoproliferative disorder of skin. In contrast, patients who present with a distinct mass may have advanced stage or possibly systemic disease and have a poorer prognosis.     

Anticancer effect of sirolimus in renal allograft recipients with de novo malignancies

The inhibition of mTOR is a target for anticancer drugs in posttransplant malignancies. The influence of conversion to sirolimus after malignancy diagnosis was investigated on patient and renal allograft survivals. The 20 renal allograft recipients (4 women, 16 men) of ages 26 to 73 years (mean, 59 years) developed malignancies within 6 to 172 months (mean, 53 months) after transplantation. Three patients developed posttransplant lymphoproliferative disease (PTLD); four, Kaposi sarcoma, three, lung cancer; two, malignant melanoma; two, breast cancer; two, renal cell carcinoma; one, Merkel cell carcinoma; one, cutaneous T-cell lymphoma; one, larynx cancer; and one, gingival cancer. After tumor diagnosis, calcineurin inhibitors, azathioprine, or mycophenolate mofetil (MMF) were discontinued abruptly and sirolimus introduced (2 mg/d; target trough level, 4.0 to 8.0 ng/mL). Prednisone was maintained. The observation time of sirolimus therapy was 4 to 48 months (mean, 14 months). Two patients with PTLD (large B-cell lymphoma) and four with Kaposi sarcoma had full regressions. Eleven patients (larynx cancer, melanoma, breast cancer, T-cell lymphoma, renal cell carcinoma, Merkel cell carcinoma, and skin lymphoma) in addition to sirolimus therapy, underwent oncologic treatment, namely, surgery and/or chemotherapy. Six patients died from disseminated malignancy 4 to 9 months after conversion. One patient with T-cell lymphoma lost his graft; in the remaining patients, serum creatinine level was stable. In conclusion, Conversion to sirolimus resulted in regression of large B-cell lymphoma and Kaposi sarcoma. In patients with advanced or disseminated malignancy, the tumors progressed. Graft function was preserved after conversion to sirolimus.     

Ki-1 positive large cell lymphoma. A morphologic and immunologic study of 19 cases

In this report, we analyze the clinical, morphologic, and immunologic findings of 19 patients with Ki-1+ large cell lymphoma, a recently described malignant lymphoma which is usually of T-cell phenotype. Most patients in this selected series of 14 children and adolescents and five adults presented with peripheral lymphadenopathy or skin lesions. No patient had bone marrow involvement. Distinctive morphologic features of Ki-1+ large cell lymphoma are tumor cell pleomorphism, sinus infiltration, fibrosis, partial lymph node involvement, sparing of B-cell regions, and a prominent plasma cell infiltrate. Seventy-two percent of cases were of T-cell phenotype; the remaining cases expressed neither B- nor T-cell-specific markers. Virtually all cases were positive for Ia (HLA-DR), Tac (interleukin-2 receptor), and T9 (transferrin receptor), indicating that this lymphoma is frequently a tumor of activated T-cells. We conclude that a diagnosis of Ki-1+ large cell lymphoma should be considered in any pleomorphic tumor with the features described in this report.     

Laparoscopy in the management of pediatric intraabdominal tumors.

BACKGROUND AND OBJECTIVES: Unresectable intraabdominal tumors pose a challenge to the pediatric oncology team. Tumor tissue is needed for diagnostic and prognostic analyses. Laparotomy is quite invasive and computerized tomography-guided needle biopsies, though less invasive, may not procure enough tissue for a complete analysis. This study aimed to evaluate our experience with laparoscopy in managing these tumors. METHODS: Nine children underwent laparoscopy: 6 for primary tumor biopsy, 2 for tumor resection, and 1 for tumor dissection followed by laparotomy for tumor resection. RESULTS: The study included 7 females and 2 males with a median age of 5 years (range, 3 to 15 years). Three patients had neuroblastoma, 2 had mature ovarian teratomas, 2 had rhabdomyosarcoma, 1 had erythrophagocytic histiocytosis, and 1 had T-cell hepatosplenic lymphoma. In each case, adequate tissue was procured for diagnostic and prognostic analyses. No perioperative complications occurred. Both patients with ovarian teratomas are well. Four patients continue to receive anti-cancer therapy. The patient with erythrophagocytic histiocytosis underwent a cord-blood stem cell transplant. One patient with neuroblastoma was lost to follow-up, and 1 patient with rhabdomyosarcoma died of progressive disease. CONCLUSIONS: Laparoscopy is a valuable technique in the management of pediatric intraabdominal tumors. It allows for tumor biopsy under direct vision, and adequate tissue is procured for all analyses. Moreover, it allows the surgeon to dissect the tumor and determine resectability.     

Primary non-Hodgkin's lymphoma of the bile ducts mimicking cholangiocarcinoma

BACKGROUND: Primary non-Hodgkin's lymphoma (NHL) of the liver and bile duct mimicking cholangiocarcinoma is rare. METHODS: The clinical and radiologic features and the treatment of 2 patients with primary NHL of the bile ducts are presented and analyzed together with cases collected from a review of the English literature between 1966 and 2003. RESULTS: Fifteen patients with primary NHL, including our 2 patients, presented with clinical features mimicking cholangiocarcinoma. All had jaundice; 9 had systemic symptoms; 7 had abdominal pain; and 5 had mass lesions. All had biliary strictures as shown on cholangiography. Two patients were infected with human immunodeficiency virus-1. In only 1 patient was the diagnosis established without surgery. Immunophenotyping in 10 patients showed 9 B-cell tumors and 1 T-cell tumor. Twelve patients underwent resection. Seven received chemotherapy immediately after the diagnosis was made. Only 3 patients have survived more than 3 years, with the longest survival being 68 months. CONCLUSIONS: Non-Hodgkin's lymphoma of the liver and bile duct must be considered in the differential diagnosis of patients with obstructive jaundice. If the correct diagnosis is made before surgery, current protocols of chemotherapy may be the primary modality of therapy. Surgical resection should be reserved to address complications of biliary obstruction or the failure of chemotherapy to eradicate localized disease.     

Mantle cell lymphoma involving skin: cutaneous lesions may be the first manifestation of disease and tumors often have blastoid cytologic features

We describe five cases of mantle cell lymphoma involving skin. Three patients initially presented with skin lesions but had evidence of widespread disease at time of diagnosis or with relatively short follow-up. One patient was known to have disseminated disease before he developed skin lesions. One patient presented with a solitary skin nodule on the thigh and has developed multiple smaller nodules on the same leg, but no other sites of disease over 30 months of clinical follow-up. This case fulfills the criteria for primary cutaneous lymphoma as proposed by the European Organization for Research and Treatment of Cancer. Biopsy of the skin lesions in all cases showed predominantly dermal and focally subcutaneous lymphoid infiltrates, preferentially perivascular and periadnexal in four cases, and nodular in one case. The tumors were composed of small- to medium-sized lymphocytes with irregular nuclear contours. Four cases had blastoid and one case had typical cytologic features. Immunophenotypic studies showed that all cases were positive for CD20 and cyclin D1, and four of five were positive for CD5. Four cases, including the CD5-negative case, had evidence of the t(11;14) shown by either fluorescence in situ hybridization methods performed on skin tumors or conventional cytogenetic analysis performed on involved bone marrow. We conclude that mantle cell lymphoma can involve skin, usually as a manifestation of disseminated disease, and is often associated with blastoid cytologic features. Rare cases of mantle cell lymphoma may arise in skin.     

Sinonasal lymphoma: a clinicopathologic analysis of 58 cases from the Massachusetts General Hospital.

Few large series compare lymphomas of the nasal cavity with those of the paranasal sinuses. We studied the cases of 58 patients, 34 males and 24 females, aged 7 to 92 years (mean, 57 years), who had lymphoma involving the nasal cavity or paranasal sinuses. Thirty-three patients had diffuse large B-cell lymphoma (DLBCL). Twenty-three were male and 10 were female, with an age range of 7 to 91 years (mean, 63 years); two were HIV-positive. Only 2 of 11 cases tested (one in an HIV-positive patient and one of lymphomatoid granulomatosis type) were Epstein-Barr virus (EBV)-positive. Thirty (91%) involved paranasal sinuses, 10 with nasal involvement, whereas three cases had nasal, but not sinus, involvement. At last follow-up, 16 (67%) were free of disease 7 to 169 months later (mean, 65 months), and 8 (33%) had died of disease 2 to 166 months later (mean, 45 months). Seventeen patients had nasal-type natural killer (NK)/T-cell lymphoma. There were 10 women and 7 men, aged 27 to 78 years (mean, 48 years). Thirteen of 14 were EBV-positive. Sixteen patients had nasal involvement, eight with sinus involvement. Eleven (73%) of 15 were alive and well 6 to 321 months later (mean, 139 months), three (20%) died of lymphoma 1, 11, and 12 months later, and one (7%) is alive with disease. There was one case each of marginal zone B-cell lymphoma, Burkitt's lymphoma, Burkitt-like lymphoma, peripheral T-cell lymphoma of unspecified type, and adult T-cell lymphoma/leukemia. In an additional three cases, the lymphomas were composed predominantly of large cells, but no immunophenotyping could be performed for subclassification. In 19 cases (17 DLBCLs, 1 Burkitt-like lymphoma, and 1 lymphoma of uncertain lineage), presenting symptoms included complaints related to the eyes. In 16 cases (13 DLBCLs, 1 Burkitt-like lymphoma, 1 nasal NK/T-cell lymphoma, and 1 lymphoma of uncertain lineage), the orbit was invaded by lymphoma. In our series, the most common lymphoma to arise in the sinonasal area is DLBCL, followed by nasal NK/T-cell lymphoma. Comparison of these two types of lymphoma showed that lymphomas involving sinuses without nasal involvement were predominantly DLBCLs (20 of 21), whereas nasal cavity lymphomas without sinus involvement were usually NK/T-cell type (8 of 11) (p = 0.000125). Compared with patients with DLBCL, patients with nasal NK/T-cell lymphoma were overall younger, with a lower male-to-female ratio. Lymphomas of B-cell lineage were more likely to be associated with symptoms related to the eyes (p < 0.0005) and to have extension to the orbit (p < 0.01) than were lymphomas of T- or NK-cell lineage. In contrast to results of Asian studies in which nasal NK/T-cell lymphoma has a very poor prognosis, our nasal NK/T-cell lymphomas had an outcome similar to that of DLBCL.     

T-cell lymphoblastic lymphoma with eosinophilia associated with subsequent myeloid malignancy.

Three patients with T-cell lymphoblastic lymphoma and peripheral blood eosinophilia are reported. At the time of diagnosis, all patients had lymphadenopathy, and one had a mediastinal mass. Lymph node biopsies revealed lymphoblastic lymphoma admixed with a variable number of mature eosinophils. Immunophenotypic studies demonstrated that each lymphoma had an immature T-cell immunophenotype. Bone marrow biopsies were hypercellular with myeloid hyperplasia and eosinophilia but were negative for lymphoma. All patients received multiagent chemotherapy; one patient achieved a complete remission, and two patients had partial remissions. All patients subsequently developed a myeloid malignancy. Two died of acute myeloid leukemia within 18 months of the diagnosis of lymphoblastic lymphoma. The third patient relapsed with a lymphoma that had histologic and immunophenotypic features of both T-cell lymphoblastic lymphoma and granulocytic sarcoma and also developed a poorly defined myeloproliferative disorder. These findings suggest that T-cell lymphoblastic lymphoma associated with eosinophilia may represent a distinct clinico-pathologic entity with a high risk of subsequent myeloid neoplasia.     

Urogenital tumors and HIV-1 infection

Four patients with solid genito-urinary tumors and HIV 1 infection have been treated in our institution over the last 2 years, including 2 with seminoma, 1 with renal adenocarcinoma and 1 with renal angiosarcoma. All had severely impaired immunity with a low CD4 level. Three had or developed a true AIDS syndrome according to the WHO and CDC criteria (1988). The remaining patient was seropositive and died less than 3 months following the diagnosis of renal angiosarcoma, he is the first reported case of renal sarcoma in a patient infected with HIV 1. Two patients were homosexuals and the other 2 were drug addicts. Along with other reported cases, our cases may underline the association between depression of immunity due to HIV and onset of solid genito-urinary tumors.     

Renal cell carcinoma in solitary kidneys

We report 10 cases of renal tumors in a solitary kidney that were treated surgically with efforts to spare renal function. Three patients were rendered anephric to excise completely the renal cancer and 2 remain without disease. The remaining 7 patients had complete tumor excision with the aid of partial nephrectomy. One patient had bilateral oncocytomas. Of the 6 partial nephrectomy patients with cancer 1 (18 per cent) remains free of metastatic disease. Concern is expressed for the role of partial nephrectomy as curative surgery for renal cancer.     

Deoxyribonucleic acid content and medroxyprogesterone acetate treatment in metastatic renal cell carcinoma

High dose medroxyprogesterone treatment was given to 20 patients with metastatic renal cell carcinoma. Distant metastases occurred before the perifascial nephrectomy in 11 patients and following nephrectomy in 9. Tumor deoxyribonucleic acid content was analyzed by flow cytometry in 8 fresh samples from each primary tumor. Four patients had homogeneously diploid primary tumors, 5 had tumors with diploid and aneuploid samples, and all 8 tumor samples were aneuploid in 10 patients. Deoxyribonucleic acid analysis was unsuccessful in 1 patient. One patient with a diploid primary tumor died of an intercurrent disease. Three patients (16 per cent) had objective remissions and 1 had a long-lasting stable disease. Of the 4 patients with any response to medroxyprogesterone acetate treatment 3 had diploid primary tumors, and 1 had 8 diploid and 2 aneuploid samples in the primary tumor. The remaining 14 patients showed no response to treatment and had progressive disease (11 of these patients died within 14 months). All 14 patients had aneuploid primary tumors. The results indicate that tumor ploidy might be related to response to medroxyprogesterone acetate treatment. Deoxyribonucleic acid content seems to be an important parameter to consider in planning treatment of metastatic renal cell carcinoma.     

CD20+ T-cell lymphoma: clinicopathologic analysis of 9 cases and a review of the literature

Rare cases of CD20+ T-cell lymphoma (TCL) have been reported, but the clinicopathologic spectrum of this disorder is not known. We identified 9 cases of CD20+ TCL diagnosed at our institution and 26 additional cases through a search of the English language literature. Among current cases, there were 7 men (ages 71 to 81, median 75 y) and 2 women (ages 36 and 37 y). Five patients presented with predominantly nodal disease (localized in 3 and widespread in 2 cases) and 4 patients presented with purely extranodal disease involving the parotid glands, skin, or small intestine. CD20 was uniformly and strongly expressed in 5 cases and dimly expressed or present on a subset of neoplastic cells in 4 cases. The proportion of CD20+ T cells changed over time in 3 cases. Three cases fulfilled diagnostic criteria for clinicopathologically defined subtypes of TCL (2 mycosis fungoides; 1 enteropathy-type TCL), whereas 6 were peripheral TCL unspecified with variable cytomorphology, T-cell immunophenotype, and sites of involvement. In 8 of 9 cases, a clonal T-cell population was identified by molecular genetic analysis. Among 8 cases with clinical follow-up, 5 behaved aggressively with death from disease within 3 years of diagnosis in 4 cases (median survival: 11 mo, range: 1 to 35 mo), and recurrent disease at 10 months in 1 case; 1 patient died of an EBV+ B-cell lymphoma (BCL) 66 months after the original diagnosis; in the remaining 2 cases, patients were alive and undergoing treatment (follow-up: 4 and 18 mo). Historical cases showed similar clinicopathologic variability. CD20+ TCL is rare, and clinically and pathologically heterogeneous. When CD20 expression is present in TCL, it may be dimmer than that of normal B cells, suggesting neoplastic transformation of a normal CD20dim+ T-cell subset. Cases of CD20+ TCL in which the proportion of CD20+ cells changes over time may reflect aberrant expression of CD20, possibly as an activation marker, by neoplastic T cells. CD20+ TCL may cause diagnostic difficulty, particularly in cases that clinically and pathologically mimic BCL. Knowledge of the unusual phenomenon of CD20 expression in TCL, in conjunction with careful morphologic analysis, the use of a panel of antibodies, and molecular genetic studies, is important in avoiding a misdiagnosis of BCL.     

Most nasal/nasopharyngeal lymphomas are peripheral T-cell neoplasms

Eleven consecutive cases of nasal/nasopharyngeal lymphoma were studied histologically and with a panel of monoclonal antibodies. The disease showed a male predominance and occurred over a wide age range, with a median of 52 years. Five patients had midfacial destructive disease, and six had gross tumor masses involving the nose/nasopharynx. Three cases were classified as small cleaved cell, two cases as mixed cell, five cases as large cell, and one case as immunoblastic lymphoma. Those belonging to the first two categories also satisfied the diagnostic criteria of so-called polymorphic reticulosis. Epithelial invasion, angioinvasion, and coagulative necrosis were demonstrated in seven, eight, and nine cases respectively. Immunohistochemical studies confirmed that the atypical cells of all 11 cases exhibited T-cell markers. All but one case had lost one or more T-cell markers associated with peripheral T cells, particularly T1 and T3. Loss of T-cell markers in the five cases histologically consistent with polymorphic reticulosis provided further support to indicate that the lesion was neoplastic. A significant proportion of cases also expressed the activated T-cell markers 12 (HLA-DR) and interleukin-2 receptor (IL-2R1).     

Beta-HCG aberrant expression in primary mediastinal large B-cell lymphoma.

We report on a primary mediastinal large B-cell lymphoma with aberrant expression of beta-human chorionic gonadotropin (beta-hCG). The patient, a 33-year-old man, had cough, dyspnea, fever, superior vena cava syndrome, and a mediastinal bulky tumor. A biopsy showed that the latter was characterized by large cells, sclerosis, and compartmentalization. The neoplastic elements expressed CD45, CD20, CD79a and, partially, CD30, whereas they were negative for CD3, epithelial membrane antigen and cytokeratins. Surprisingly, they displayed a clear-cut positivity for beta-hCG. The remaining oncofetal markers applied (PLAP and alpha1-fetoprotein) were negative. Electron microscopy demonstrated the presence of numerous nuclear pockets and the lack of intercellular junctions. DNA analysis by polymerase chain reaction showed clonal rearrangement of Ig heavy-chain genes. The patient responded promptly to the administration of MACOP-B. To the best of our knowledge, this is the first example of B-cell lymphoma showing positivity for beta-hCG; a similar aberrant expression was previously observed only in three Japanese patients with human T-cell lymphotropic virus type I+ adult T-cell lymphoma/leukemia. Because primary mediastinal large B-cell lymphoma has in the past been frequently confused with germ cell tumors, pathologists should be aware of possible beta-hCG expression by lymphomatous cells to avoid the risk of misdiagnosis.     

Primary extranodal soft tissue lymphoma of the extremities

Since true primary lymphomas of soft tissues are extremely rare and there are few well-documented cases in the literature, we report eight cases to help define the clinical and pathologic features of this type of extranodal lymphoma. Among 7,000 malignant lymphomas seen at Mayo Clinic over a 10-year period, we found eight cases of Stage IAE extranodal malignant lymphoma that presented as a soft tissue mass in the upper or lower extremities. The tumors occurred in the following sites: thigh (n = 4), upper arm (n = 2), ankle (n = 1), and calf (n = 1). Two cases had low-grade histology: small lymphocytic lymphoma and follicular mixed small-cleaved and large-cell lymphoma. An intermediate-grade histology was present in two cases: diffuse mixed small- and large-cell lymphoma and diffuse large-cell (cleaved) lymphoma. Half of the patients had a high-grade lymphoma: diffuse large-cell (n = 3) and large-cell, immunoblastic (n = 1). The results of immunohistochemistry and electron microscopy in selected cases are discussed in the context of the differential diagnosis with other small, round-cell cancers. Four patients died of recurrent lymphoma, one at 7 months, two at 15 months, and one at 4 years. Three patients are alive without evidence of disease at 3, 8, and 13 years. One patient is alive and is receiving chemotherapy for locally recurrent lymphoma 6 months after diagnosis.     

Malignant thymoma in children: a 20-year review

Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 2 1/2 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.     

Combined thoracic aortic or upper digestive tract resection for lung cancer and malignant mediastinal tumor

Objective: We studied possible indications and combined resection in patients with lung cancer and mediastinal tumors requiring combined thoracic aortic or upper digestive tract resection.Methods: Ten patients with lung cancer and malignant mediastinal tumors (9 men and 1 woman aged 39 to 72 years; mean: 60.5) underwent combined aortic or upper digestive tract resection.Results: Fiv — 3 with primary lung cancer, 1 with thymic cancer, and 1 with liposarcoma —, underwent combined aortic resection. In 2 each, lung cancer and malignant mediastinal tumor had infiltrated the thoracic aorta. The remaining case of lung cancer was complicated by aortic aneurysm in the distal arch. Cardiopulmonary bypass was conducted in 4, and selective cerebral perfusion in 2. Three patients are alive after 11, 22, and 61 months without disease recurrence. Those undergoing combined upper digestive tract resection all had lung cancer, with 4 having tumors infiltrating the esophagus or corpus ventriculi. The remaining patient had both lung and esophageal cancer. The patient treated with combined corpus ventriculi resection has survived 24 months and the patient treated with combined esophageal resection has survived 12 months without disease recurrence. The 1-year survival rate was 60%, 2-year 23%, and 3-year 23%. Prognosis was generally poor with the longest survival 13 months with N2 lung cancer.Conclusions: In combined resection due to malignant mediastinal tumor, T4N0-1 lung cancer, or diseases such as aortic aneurysm, prognosis can be expected to improve. Despite the often poor prognosis in T4N2 lung cancer, surgical intervention may be indicated to avoid complications due to tumor invasion and to lengthen survival and improve quality of life.     

Bilateral malignant lymphoma of the breast in patients with a familial history of cancer: A report of three cases

We report three cases of bilateral malignant lymphoma of the breast in patients with a familial history of cancers. All the patients had B-cell malignant lymphoma and were treated by a radical mastectomy and polychemotherapy. Bilateral tumors were detected synchronously in one case, whereas the second tumors in the other cases occurred 4 years and 8 years after the initial treatment. Although the patients had no relevant past history, these cases were notable for the fact that all were associated with a familial history of cancers. One patient had a definite familial history of breast cancer, that is to say, her mother, sister and brother all suffered from this disease. The brother of another patient died of gastric cancer while two of her uncles each suffered from sarcoma of the lower extremities and a brain tumor, respectively. As for the remaining patient, one of her maternal uncles and an aunt also died of prostate cancer and leukemia, respectively. However, only one out of seven patients with unilateral malignant lymphoma of the breast used for comparison had a familial history of cancer. A possible relationship between bilateral primary malignant lymphoma of the breast and a familial history of cancer is suggested.