Barbara M. Osborne

Primary lymphoma of the liver ten cases and a review of the literature

Ten adult white patients (21–75 years old; six women, four men) presented with large cell lymphoma of the liver. Each complained of abdominal pain and/or an abdominal mass, and five had B‐symptomatology of weight loss, fever (one patient), and night sweats (three patients). At laparotomy (eight patients) or by computerized tomography, liver‐spleen scan and lymphangiogram (two patients with percutaneous liver biopsy only), the liver was the sole site of involvement, although subsequent staging procedures revealed bone marrow involvement in three patients. Initial biopsy interpretation was incorrect in four cases which were diagnosed as poorly differentiated carcinoma. Although uncommon, the differential diagnosis of primary liver lymphoma should be entertained when patients present with solitary (three cases) or multiple (six cases) liver defects, particularly when alpha‐fetoprotein and carcinoembryonic antigen levels are normal. One patient had diffuse hepatomegaly. Treatment included biopsy (eight patients) or resection (two patients) followed by combination chemotherapy. All patients are alive from 0 to over 10 years (mean, 2.4; median, 1.8 years): six in complete remission, two with less than 6‐months follow‐up, and two with recurrent lymphoma. Examination of this group of patients along with 19 cases identified in the literature suggests that this is a more treatable disease than primary liver carcinoma.

Stages IE and IIE non-Hodgkin's lymphomas of the stomach: Comparison of treatment modalities

Seventy‐nine patients with Stages IE and IIE non‐Hodgkins lymphomas of the stomach were treated between 1953 and 1980. The histopathologic classification was as follows: diffuse large cell, 61 (of which 23 were immunoblastic sarcomas [plasmacytoid]); diffuse well‐differentiated lymphocytic, 6; diffuse mixed, 1; undifferentiated non‐Burkitts, 1; nodular, 9; and unclassifiable, 1. Thirty‐five patients had Stage IE disease and 44 had Stage IIE. Treatment modalities included surgery, radiotherapy, chemotherapy, and combinations thereof. Sixty‐six patients had a laparotomy for diagnosis and/or management. Of these, only 42 had a gastrectomy. The stomach was considered to be unresectable in the other 24 patients. There were 5 postoperative deaths among 31 patients who had a laparotomy or gastrectomy at our institution. The overall 5‐year actuarial survival was 56%; the disease‐free survival was 54%. For patients with Stage IE disease the survival was 76%, and for those with Stage IIE, 42%. Promising results were obtained in 13 patients who were treated on a multimodality program consisting of four cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus bleomycin (Bleo), which was alternated with involved field radiotherapy. All 13 patients had no evidence of disease as of this report. Only one patient had a relapse (Waldeyers ring), and he was salvaged with radiotherapy. Six of these 13 were diagnosed by endoscopic biopsy and did not have a laparotomy, 3 were found to have unresectable disease at laparotomy, and 4 had a resection. Biopsy with the flexible fiberscope and treatment with CHOP‐Bleo and radiotherapy can avoid the morbidity and mortality of gastrectomy.

Primary lymphomas of the nasal cavity and paranasal sinuses

Extranodal lymphomas which present in the nasal cavity and/or the paranasal sinuses are rare. Thirty‐eight patients with disease that was clinically limited to the head and neck (Ann Arbor Stages IE‐IIE) were admitted between 1947 and 1983. Twenty‐eight patients were treated with radiotherapy alone and 10 received combination chemotherapy in addition. The overall 5‐year survival figure was 56%. The corresponding result for Stage IE was 67%. No patient with Stage IIE disease survived 5 years. Extent of the extranodal disease also influenced results for Stage IE patients who were treated with radiotherapy only. When the extranodal disease was staged using the American Joint Committee TNM system, the 5‐year disease‐free survival for T1 and T2 patients was 78% as compared with 19% for patients with T3 and T4 disease. The addition of combination chemotherapy improved results for patients with T3 and T4 lesions.

Primary splenic presentation of malignant lymphoma and related disorders: a study of 49 cases

The diagnosis of malignant lymphoma presenting as an initial splenic manifestation may go unrecognized as such when peripheral lymph nodes are not enlarged and when results of bone marrow biopsies are negative. Tissues from 49 patients, ranging in age from 15 to 78 years, in whom the original diagnosis of malignant lymphoma and related conditions was made at splenectomy, were classified as: diffuse small lymphocytic (20), diffuse large cell (ll), diffuse small cleaved (5), diffuse large cell, immunoblastic (5), follicular small cleaved cell (3), and follicular mixed small cell and large cell (2). Two additional spleens, diagnosed as acute blastic leukemia, were initially confused with malignant non‐Hodgkins lymphoma by light microscopy. One patient presented with Hodgkins disease confined to the spleen. For the non‐Hodgkins lymphoma group, parameters of age, sex, splenic weight (range, 226‐4000 g), lymph node, bone marrow, or liver involvement did not adversely influence prognosis. Abdominal lymph nodes were positive in 31 of 37 patients having splenic hilar and/or abdominal lymph nodes available for review. Of 29 patients with adequate follow‐up, 7 died of disease, 5 were free of disease at 3 years, 2 were free of disease at 5 years, 2 were alive with disease at 3 years, 4 were alive with disease at 5 years, and 9 died from second malignancies, unknown, or unrelated causes. Six of the 7 patients who died of lymphoma were classified as large cell (four diffuse large cell and two diffuse large cell, immunoblastic), with a mean 2‐year survival. One patient died of leukemia. Those lymphomas classified as low grade behaved in an indolent fashion. The morphologic diversity of these cases emphasizes the need for the initial recognition and correct classification of lymphomas which present in the spleen, since survival is best determined according to histologic type.

Extrarenal Wilms tumor

The previous reports of extrarenal Wilms tumor are reviewed and two additional patients presented. The clinical and pathologic features are discussed. The diagnosis of extrarenal Wilms tumor does not imply a bad prognosis. Treatment should utilize multidrug chemotherapy similar to the guidelines set by the National Wilms Tumor Study.

Residual fibrous masses in treated Hodgkin's disease

Of nine patients with residual masses following therapy for Hodgkins disease (HD), eight had nodular sclerosing HD, and one had mixed cellularity HD. One patient had Stage II disease, seven had Stage III, and one had Stage IV. Seven patients presented with bulky mediastinal disease. Regardless of the initial therapy used residual masses in the mediastinum and/or peripheral locations stabilized in 1 to 8 months. Between 5 and 10 months after initiation of therapy, five patients underwent resection of mediastinal or paratracheal masses; three patients had resection of peripheral masses, and one patient underwent laparatomy. Microscopically, the resected masses were hyalinized tissue showing a characteristic nodular configuration without evidence of active HD. Stable residual mass lesions occurring after therapy for HD should not be assumed to represent recalcitrant malignancy, as they may show only fibrosis.

Recurring Congenital Lesion of the Cheek

A 3-cm purple area was present on the left cheek of a newborn white female, and a biopsy showed atypical cells in fibroadipose tissue. The lesion increased in size and the infant was referred to the M. D. Anderson Hospital. By 6 weeks, the lesion formed an irregular 6-cm paranasal mass, compressing the nostril and depressing the corner of the mouth. It was soft and mobile with a nodular, erythematous, excoriated surface. Three small satellite masses were also present. Another biopsy was obtained, establishing the presence of a malignant neoplasm. Vincristine, adriamycin, and cyclophosphamide produced a rapid decrease in size of the lesion, and by 6 months it was considerably smaller and firmer. Total surgical excision was then performed. The well-circumscribed 3-cm nodule had a glistening, tan-gray cut surface. The wound was closed with a skin graft, andBy 11 months, the lesion had recurred as a firm, round mobile nodule in the premaxillary area, lateral to the previous surgical site. The reexcision speci...

Unexpected splenic nodules in leukemic patients

We reviewed a series of five splenectomy specimens from patients with various leukemias (three cases of chronic myelocytic leukemia, one case of acute myelocytic leukemia, and one case of hairy cell leukemia). In addition to diffuse red pulp disease, we unexpectedly encountered nodules grossly in each of the specimens. These represented sea blue histiocytosis (one case), focal hairy cell leukemia (one case), localized blast transformation (one case), and concentrated foci of treated leukemia (two cases), with a prominence of immature granulocytic precursors on a background of trilineage hyperplasia. These cases are reported because they are unusual and because they furnish interesting correlates of gross and microscopic anatomy.

The Quarterly Case:Inguinal Lymphadenopathy in a 52-Year-Old Male

CASE HISTORYA 52-year-old Latin American male saw his physician becaucae of swelling of the right thigh, which he dated to an automobile accident that had occurred 3 years earlier. He also com-plained of skin eruptions following exposure to cold or emotionally stressful situations and of a 30-pound weight loss.