Malignant lymphoma of the testis

Lymphoma presenting in the testis was evaluated in 24 patients. The tumours were classified according to both the Rappaport and the Kiel classification. The Rappaport classification yielded only two tumour types--15 diffuse histiocytic and 9 diffuse mixed histiocytic-lymphocytic, with no pure lymphocytic lymphoma. No difference in survival time was found between these two tumour types. According to the Kiel classification there were four histological tumour types--immunoblastic (11 cases), centroblastic (2), centroblastic/centrocytic (6) and polymorphic immunocytic (5). In comparison with the other tumour types, the centroblastic/centrocytic lymphomas seemed to be advantageous as regards survival. It is concluded that systemic treatment with chemotherapy in accordance with current principles for malignant lymphoma is indicated following orchiectomy in localised testicular lymphoma as well as in disseminated disease. The value of post-operative radiotherapy to regional nodes is questionable.     

Lymphoma of the breast.

Thirteen patients with lymphoma of the breast are presented. In addition, 163 previously reported cases of lymphoma of the breast are reviewed. Complete staging was performed on all patients. No patient had a diagnosis of lymphoma prior to breast biopsy. The histologic findings were diffuse histiocytic lymphoma (DHL) in eight patients, nodular lymphocytic poorly differentiated lymphoma (NLPD) in two patients, nodular mixed lymphoma (NM) in two patients and nodular sclerosing Hodgkin's disease (NSHD) in one patient. Five patients had Stage IV disease, two had Stage III disease, four had stage II disease and two Stage I disease. Nine patients each underwent an excisional biopsy and four patients each had a modified radical mastectomy as initial therapy. Two patients each underwent a staging laparotomy. In advanced disease, chemotherapy achieved complete remissions in approximately 50% of patients. Unfavorable histologic findings are most common in lymphoma of the breast and thorough staging is necessary to select the best form of therapy. The absolute survival rate (61%) and the disease free survival rate (46%) are similar to nodal lymphoma of corresponding histologic factors and stage.     

Surgical management of lymphomas involving the chest

The efficacy of surgical judgments in the management of thoracic lymphoma was studied through review of 34 patients with primary mediastinal lymphomas, 30 patients who needed one or more thoracic operations after treatment of extrathoracic lymphomas, and 5 patients with primary lymphocytic infiltrates (PLI) of the lung. In all patients with primary lymphocytic infiltrates of the lung, thoracotomies were required in order to establish the correct diagnosis. Patients with primary mediastinal lymphoma required 74 surgical procedures (2.2 per patient) to establish the correct diagnosis. In retrospect, 40 operations were not beneficial. The procedures that provided diagnostic samples were 15 anterior mediastinotomies, 8 full thoracotomies, 3 median sternotomies, and 8 lesser procedures such as mediastinoscopy. Patients who needed thoracic procedures after treatment of extrathoracic lymphomas had 41 thoracic operations (1.37 per patient). Five operations were needed to manage complications of thoracic lymphoma or its therapy. To evaluate new radiographic findings, there were 35 operations (1.3 per patient) at a mean interval of 5.0 years following initial treatment; the findings were recurrent lymphoma in 62% and new lesions in 38% of patients. Among new lesions, there were 2 bronchogenic cancers; 9 enlarging mediastinal-pleural masses were not caused by lymphoma. The accurate diagnosis of thoracic lymphoma or new thoracic lesions in patients with lymphomas usually requires enough tissue for immunophenotyping. Providing adequate tissue samples and treating new lesions that are not lymphomas often require major thoracomediastinotomies for immunophenotyping.     

Malignant lymphoma of the thyroid gland: A clinicopathologic study

This study reviews 21 cases of thyroid lymphoma diagnosed between 1969 and 1980. The thyroid gland was the primary site in all but two cases. The 20 patients with non-Hodgkin lymphoma had a mean age of 66 years, 75% were women, and all were Caucasian. A rapidly growing thyroid mass with hoarseness, dysphagia, and difficulty in breathing was the initial finding. Most patients had diffuse histiocytic lymphomas. Associated Hashimoto thyroiditis was found histologically in 57% of the cases. Using the immunoperoxidase stain method on tissue sections, intracytoplasmic monoclonal immunoglobulin was demonstrated in 5 of 15 cases. All but two patients received radiation therapy, and 13 received chemotherapy. The median survival was 8 months; 20% died of unrelated causes and 32% are alive (average duration: 27 months). Dissemination, after local control by radiation therapy, was the leading cause of death. The prognosis was better in men, in patients under the age of 65 years, and in those patients who, on biopsy, did not have involvement of the extrathyroid soft tissue or regional lymph nodes.     

Primary lymphoma of the large intestine

Three cases of primary colonic lymphoma are presented and the literature for this disease is reviewed. Colonic lymphoma occurs in from 0.5 to 2 per cent of all cases of neoplastic disease of the colon, and comprises 15 to 20 per cent of cases of primary gastrointestinal lymphoma. The disease can occur at any age, from 3 years to 89 years; the average age is 50 years. Men are affected twice as often as women. The disease most commonly presents as a painful abdominal mass. The cecum is the area of involvement in 70 per cent of cases. The tumors are usually large, averaging over 7 centimeters. The most common histological types are histiocytic or lymphocytic tumors. Surgery is the principle treatment mode; about half are thought to be potentially curable at the time of surgical exploration. The role of adjuvant therapy has not yet been clearly defined, although data from some studies indicate increased survival in those patients given adjuvant radiation therapy. Survival is decreased in patients with tumors larger than 5 centimeters or with histiocytic cell type. Overall 5-year survival is 39 per cent.     

High-dose methotrexate for non-AIDS primary central nervous system lymphoma. Report of 13 cases

Thirteen patients with primary lymphoma of the central nervous system (CNS) were treated with high-dose intravenous methotrexate (MTX), 3.5 gm/sq m, followed by calcium leucovorin rescue, at 3-week intervals, for three cycles. Eleven patients subsequently received radiation therapy to the whole brain, 30 to 44 Gy. Before radiation therapy, eight patients responded completely and four partially; there was one non-responder. The median Karnofsky score before high-dose MTX therapy was 60 and increased to 90 after treatment. Five of the eight complete responders reached a Karnofsky rating of 100. The three longest responders (one of whom received MTX only) were without recurrence of their disease at 29+, 32, and 32+ months posttherapy. The median response period is 9+ months. The median survival time from the date of the first MTX treatment is 9+ months, and the three longest survival times are 29+, 32+, and 54+ months. All patients received corticosteroids in either unchanging or diminishing dosages during therapy. It is concluded that primary CNS lymphoma is sensitive to high-dose MTX, which provides a safe and easily administered adjuvant to radiation therapy for this neoplasm.     

Malignant lymphoma of the testis: a clinicopathologic study of 37 cases.

The clinicopathologic findings in 37 cases of malignant lymphoma with primary clinical manifestation in the testis were analyzed. Of these cases 34 were classified as histiocytic lymphoma and 3 as lymphocytic lymphoma. Patients usually presented with a painless small testicular mass of short duration. The peak incidence occurred in the fifth through eighth decades. Metachronous testicular involvement was noted in 35 per cent of the cases and simultaneous bilateral involvement in 3 per cent. In 25 per cent of the cases the first manifestation of lymphoma after orchiectomy was in the opposite testis. Regional and distant nodes were equally common sites of lymphoma after orchiectomy. The long-term survival of a few patients after orchiectomy may suggest that some malignant lymphomas are truly primary in the testis. However, the short interval free of disease and the rapid death of most patients imply that most testicular lymphomas are a manifestation of multicentric origin of systemic malignant lymphoma. The over-all 2 and 5-year survival rates were 30 and 20 per cent, respectively. Prospective studies are needed to define the optimal modality of treatment.     

Management of primary gastric lymphoma.

A retrospective study of 51 patients treated for primary gastric lymphoma was made to evaluate the influence of clinicopathologic features and the method of treatment on survival. The lymphocytic type of tumor showed a higher survival rate than the histiocytic type. Tumors that involved only the gastric wall resulted in a five-year survival rate of 62%, as compared with 50% for tumors that involved also the adjacent gastric lymph nodes, and 25% for those involving the distant gastric lymph nodes. Treatment by subtotal, total, or extended forms of gastric resection without postoperative radiation gave a five-year survival rate of 33%. Resection followed by radiation yielded a higher survival rate of 67%. The best results were obtained in 13 patients who received higher doses of radiation: 11 (85%) survived five or more years. One-third of the patients developed manifestations of systemic malignant lymphoma after curative therapy. These observations suggest a planned multimodal therapeutic program for this disease.     

Primary gastric lymphoma. Problems in staging and management

A retrospective review of 28 patients with primary gastric lymphoma was performed to determine the optimal treatment modality. The presenting signs and symptoms resembled peptic ulcer disease or gastric carcinomas. The majority of the lymphomas were of the diffuse histiocytic subtype. There was a 75 percent resectability rate in those patients operated on. Palliative resection produced a 5 year duration of survival of 28 percent and curative resection, 43 percent. Eighteen patients underwent a subtotal gastrectomy, and a total of 10 patients presented with stage I disease. The longest median duration of survival at last follow-up was 32 months for patients with stage IB disease compared with a median duration of survival of 8 months for those with stage III disease. Adjuvant radiotherapy and chemotherapy may improve survival after a curative resection is performed if there is serosal penetration or nodal involvement.     

Surgical therapy of localized abdominal non-Hodgkin's lymphomas

Non-Hodgkin's lymphomas may involve a variety of abdominal organs, including the liver, spleen, gastrointestinal tract, and retroperitoneum. The number of organs potentially involved and the noncontiguous mode of spread make non-Hodgkin's lymphoma a difficult tumor to evaluate at the time of laparotomy. To clarify the surgical management of patients with this tumor, we retrospectively reviewed the medical records of 202 patients with histologically proven abdominal lymphomas. Within this group, 36 patients underwent laparotomy before they had chemotherapy or radiation therapy. Ten patients were explored to establish a histologic diagnosis of lymphoma. The remaining 26 patients underwent laparotomy because of presumed benign disease. Twenty patients were found to have localized disease at laparotomy. Patients with localized disease demonstrated significantly better survival than patients with extranodal and nodal involvement (p less than 0.05). Four patients with local resection received no adjuvant therapy and were free of disease a median of 50 months after surgery.     

Primary lymphoma of the breast

Primary non-Hodgkin's lymphoma of the breast accounts for fewer than 3% of extranodal lymphomas. As compared to extranodal lymphomas in other sites they are characterised by more rapid progression and a worse prognosis. The aim of the study was to investigate 5 cases of primary lymphoma of the breast and review previous studies in a search for any preoperative characteristics that could assist in the management of lymphoma of the breast. All patients (n = 5) who were diagnosed with lymphomatous involvement of the breast between 1996 and 2004 were evaluated retrospectively. All patients staged IE (breast involvement only) or IIE (limited to the breast and ipsilateral armpit) were included. Most of the primary breast lymphomas were of intermediate grade. Patients received some combination of surgery, radiation, and chemotherapy. The mean follow-up was 48 months (range 24 to 72 months). All 5 patients survived at least 6 years from the time of diagnosis. Long-term survival in patients with primary non-Hodgkin's lymphoma of the breast is possible. The clinical outcome of patients with breast lymphoma depends on the histology and appears to parallel that of patients with lymphoma of similar histology involving other sites.     

Nasal lymphoma. A clinicopathologic study with immunophenotypic and genotypic analysis

We studied 13 cases of malignant lymphoma involving the nasal cavity, in six men and seven women, from 27 to 92 years of age (mean, 56 years; median, 55 years). All lymphomas had a diffuse pattern, with 10 of large-cell type (six immunoblastic polymorphous, one immunoblastic, three large cleaved cell), one of mixed small- and large-cell type and one of small cleaved-cell type. One case could not be subclassified. Angioinvasion and prominent necrosis were seen in 10 cases. Pseudoepitheliomatous hyperplasia of the overlying epithelium was present in five cases. Immunohistochemical studies on frozen or paraffin sections in nine cases revealed that the atypical cells were T cells in four cases (CD8+ in two cases) and B cells with monotypic immunoglobulin in two cases. In three cases, the findings were suggestive but not diagnostic of T lineage. Genotypic analysis in one of two cases of T-cell lymphoma revealed clonal rearrangement of the genes for beta and gamma chains of the T-cell receptor. Patients were treated initially with local radiation therapy (10 cases) or with radiation and chemotherapy (three cases). Eight patients (62%) had no relapse and were free of disease between 9 months and 23 years (mean, 6 years 5 months; median 2 years 1 month) after diagnosis. Five patients developed recurrent disease, three of whom were successfully salvaged. One patient was alive with tumor at the time of last follow-up and one died with tumor. Among cases of malignant lymphoma presenting with involvement of the nasal cavity, we find a high proportion of angioinvasive, diffuse large-cell lymphomas, with a predominance of T-cell type, and a relatively good prognosis when treated with radiation therapy.     

Malignant lymphoma of the large intestine —Operative results in Japan

One hundred thirty cases of primary malignant lymphoma of the large intestine in Japan were studied with regard to operative results. The averaged age was 52.6 years and most of the patients were men. Sites of the tumor were the coecum (71.5 per cent), rectum (16.9 per cent) and the ascending colon (6.2 per cent). Surgery was performed for 129 (99.2 per cent) of the 130, of which 71 (55 per cent) were curatively resected. Surgical procedures were mostly Miles operation and the others were anterior resections and hemicolectomies. Postoperative 5 and 10 year survival rates were 34.8 per cent and 33.2 per cent respectively, and the rates after curative resection were 44.2 per cent and 40 per cent respectively. Prognoses were betwer when the tumor was of 5 cm or less in diameter, intraluminal and without lymph node metastasis. Classified histologically, most of the tumors were of histiocytic type, followed by lymphocytic, mixed type and Hodgkin's disease. Five and ten year survival rates of the curative resection group, by histological type, were both 38.9 per cent for histiocytic type, both 43 per cent for lymphocytic type, 43.8 per cent and 21.9 per cent respectively for mixed type, and both 100 per cent for Hodgkin's disease. Therefore, the operative result of malignant lymphoma in the large intestine was poor compared with results in case of cancer of the large intestine.     

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.     

Management of intra-abdominal lymphomas

Fifty-nine patients (mean age 65) were evaluated for intra-abdominal lymphomas, localized or disseminated, during the period between 1977 and 1985. These lymphomas were lymphocytic (66%), histiocytic (17%), follicular (10%), and mixed (7%). Liver and retroperitoneal nodes were most frequently involved (32%), followed by stomach (31%), bowel (17%), and spleen (20%). Thirty-five patients (59%) underwent some type of surgical procedure supplemented by either chemo- or radiation therapy. Curative and palliative resections accounted for 40 per cent and 26 per cent, respectively while diagnostic laparotomy with or without staging accounted for 34 per cent. The 1-month postoperative mortality was 20 per cent. The 5-year survival was 12 per cent for the surgical group and 0 per cent for the medical group (P less than 0.05). Patients with lymphomas of the stomach and spleen, treated surgically, fared better than their medical counterparts. Most patients in this series died of their disease (55%) or from attempts to eradicate it.     

Primary breast lymphoma: the role of mastectomy and the importance of lymph node status

OBJECTIVE: To construct and analyze a database comprised of all reported cases of primary breast lymphoma (PBL) that include treatment and follow-up information published during the last 3 decades. SUMMARY BACKGROUND DATA: PBL accounts for 0.4% of breast malignancies and 2% of extranodal lymphomas. Surgical therapy has varied from biopsy to radical mastectomy. Chemotherapy and radiation therapy have been used as adjuvant or primary therapy. A standard consensus treatment of PBL is not available. METHODS: We reviewed all published PBL reports from June 1972 to March 2005. A database was compiled by abstracting individual patient information, limiting our study to those reports that contained specific treatment and outcome data. Patient demographics such as survival, recurrence, and time to follow-up were recorded, in addition to surgical, radiation, and/or chemotherapy treatment(s). RESULTS: We found 465 acceptable patients reported in 92 publications. Age range was 17 to 95 years (mean, 54 years). Mean tumor size was 3.5 cm. Diffuse large cell (B) lymphoma was the most common histologic diagnosis (53%). Disease-free survival was 44.5% overall. Follow-up ranged from one to 288 months (mean, 48 months). Treatment by mastectomy offered no survival benefit or protection from recurrence. Treatment that included radiation therapy in stage I patients (node negative) showed benefit in both survival and recurrence rates. Treatment that included chemotherapy in stage II patients (node positive) showed benefit in both survival and recurrence rates. Histologic tumor grade predicted survival. CONCLUSIONS: Mastectomy offers no benefit in the treatment of PBL. Nodal status predicts outcome and guides optimal use of radiation and chemotherapy.     

Incidental and concurrent malignant lymphomas discovered at the time of prostatectomy and prostate biopsy: a study of 29 cases

The incidence and histologic features of malignant lymphomas discovered at the time of prostate biopsy, transurethral resection, and prostatectomy are not well documented. We searched our surgical pathology files for malignant lymphomas identified from prostate surgical specimens from 1989 to 2004. Of 4,831 cases of prostate specimens (3,405 biopsies, 266 transurethral resections, 1,160 prostatectomies) examined at the City of Hope during this period, 29 cases of malignant lymphomas involving the prostate and pelvic lymph nodes were identified (0.6%). These malignant lymphomas can be divided into two groups: 1) 18 incidental cases (0.37%) without prior history of malignant lymphoma; and 2) 11 cases (0.23%) with concurrent known malignant lymphoma. For the first group, the patients with pelvic node involvement ranged in age from 59 to 78 years (mean, 69.2 years; median, 70 years), and the patients with prostate involvement ranged in age from 45 to 78 years (mean, 64.6 years; median, 67.5 years). For the second group, the patients ranged in age from 53 to 80 years (mean, 66.8 years; median, 69 years). Diagnoses of all cases were confirmed by immunohistochemistry or molecular analysis. Ten of 18 cases in the first group involved pelvic nodes only, and the other 8 cases were primary prostatic lymphoma. Of 18 cases in the first group, 13 were small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL), 3 were marginal zone B-cell lymphoma, and 1 was mantle cell lymphoma. These lymphomas were not readily apparent in most cases by histologic examination, and may be confused with chronic prostatitis when the prostate was involved or with reactive lymphoid hyperplasia when pelvic nodes were involved. Immunohistochemistry and molecular studies may be necessary to confirm the diagnosis. For the second group, prostate and pelvic lymph nodes were involved as part of systemic dissemination of concurrent malignant lymphoma. The diagnosis was usually easily established in these cases. Of 11 cases, 4 were SLL/CLL, 4 were follicular lymphoma, 2 were mantle cell lymphoma, and 1 was diffuse large B-cell lymphoma.     

Oncologic outcomes of primary lymphoma of bone in adults

A retrospective analysis of adult patients who had lymphomatous involvement of bone was done to evaluate the prognostic factors and the oncologic outcome. Between 1984 and 1994, 28 patients (nine women and 19 men) were diagnosed with lymphoma of bone. The median age was 45 years (range, 23-76 years). The median followup was 40.3 months (range, 0.5 months-15.8 years). Eighteen patients (64%) were classified as having Stage IE disease, two patients (36%) were classified as having stage II disease, and eight patients were classified as having Stage IV disease. Twenty-one of the lesions were classified as diffuse large B cell with multilobulated nuclei. Two patients had local recurrence. Three patients had osteonecrosis develop at the site of their radiation therapy. The 5-year Kaplan-Meier survival estimate was 57.8% (95% confidence interval range, 40-33 82.8). Statistically improved survival was seen in patients younger than 40 years and those patients with diffuse large B cell with multilobulated nuclei. Patients with primary lymphoma of bone did not have a statistically improved survival compared with patients with systemic disease. The results of the current study suggest that age at diagnosis and histologic subtypes are important prognostic factors; however, the diagnosis of primary lymphoma of bone does not confer improved prognosis.     

Long-term follow-up after curative surgery for early gastric lymphoma.

OBJECTIVE: This study was designed to examine the long-term survival of a homogenous group of patients with stage IE or IIE-1 gastric lymphoma after complete surgical resection. SUMMARY BACKGROUND DATA: The management of gastric lymphoma remains controversial. Enthusiasm for multimodality approaches for gastric lymphoma has lead to the current trend of using chemotherapy as primary treatment, thus avoiding gastric resection. Surgery, however, may result in improved long-term survival rates. METHODS: The records of all patients with the diagnosis of gastric lymphoma from 1980 to 1991 were reviewed retrospectively. Of 106 patients examined, 34 underwent curative resection and regional lymphadenectomy for pathologically staged IE or IIE-1 (pN1) gastric lymphoma. Fifteen patients underwent surgery alone, whereas 19 also received postoperative adjuvant therapy. RESULTS: The median follow-up time was 74 months. The 10-year actuarial disease-free survival was 91% for stage IE disease (n = 23) and 82% for stage IIE-1 disease (n = 11). There were no operative deaths and a 26% morbidity rate. No difference in survival was found for those treated with adjuvant therapy. CONCLUSIONS: The results compare favorably to those reported with the use of primary chemotherapy and radiation therapy and suggest that surgery remains the best frontline therapy for early gastric lymphoma.     

Primary spinal epidural non-Hodgkin's lymphoma: report of eight patients and review of the literature.

Eight patients treated for histologically confirmed primary spinal epidural non-Hodgkin's lymphoma diagnosed between January 1979 and August 1989 (6.6% of all cases of intraspinal lymphoma) were studied. There were six men and two women. The median age was 70 years (range, 43-80 yr). Patients sought treatment for a prodrome of back pain (median duration, 3 mo) followed by an acute neurological deterioration (median duration, 6 d). The most common findings were a discrete sensory level in 5 patients, hyperreflexia in 5 patients, and paraparesis or paraplegia in 5 patients. Radiographically, there was an absence of bony destruction by these tumors. All patients underwent a decompressive laminectomy, subtotal tumor resection, and spinal irradiation (median dose, 3800 cGy). Two patients had low-grade lymphomas (one B cell and one T cell), and 6 patients had intermediate-grade lesions (six B cell). Two patients with B-cell lymphomas (one low-grade and one intermediate-grade) developed metastatic disease 15 and 17 months after the initial diagnosis; no evidence of lymphoma developed in the other 6 patients. The median survival was 22 months (range, 2-71 mo). Lymphoma was the cause of death in only 1 of the 4 patients who died, and the 4 younger patients are alive and well. Primary spinal epidural non-Hodgkin's lymphoma should be a diagnostic consideration in the older patient who seeks treatment for spinal cord compression manifested by a prodrome of back pain, followed by a rapid neurological deterioration, normal plain spine radiographs, and neuroimaging consistent with an extradural compressive lesion. Surgery for this diagnosis followed by spinal irradiation should result in significant neurological improvement.