Primary lymphoma of the gastrointestinal tract.

Primary gastrointestinal lymphoma represents approximately 1% of all gastrointestinal neoplasms. Gastric involvement is more common than small or large intestine and carries a better prognosis. Abdominal pain and weight loss may be the only manifestations and may be present for months or years before the diagnosis is made. Perforation and obstruction occur infrequently. Multiple tumors constitute 8% of cases. Although barium studies and endoscopy reveal the lesion in a high percentage of cases, exploratory celiotomy is not infrequently required for diagnosis. Only one-third of lymphomas are confined to the bowel at laparotomy. Histologically one-third are reticulum cell sarcomas and the remainder lymphosarcoma or lymphocytic lymphoma. Five year survival overall was 38%. Curative resections yielded a survival of 60% regardless of site while palliative resections offered only a 17% chance of cure. As expected, survival was inversely proportional to extent of nodal spread. Postoperative radiotherapy is recommended for residual disease.     

Primary lymphoma of the gastrointestinal tract

BACKGROUND: A retrospective analysis of 12 years' experience (1987-1999), concerning gastro-intestinal lymphomas (LNH-GI) has been performed, to offer a contribution for the identification of the diagnostic and prognostic criteria. METHODS: During this period, 11 cases (9 male and 2 female) have been treated: 8 with gastric localization and 3 with intestinal localization, with age ranging between 12 and 78 years. Most of the analyzed cases (5 cases out of 8 with gastric localization and all the cases with intestinal localization) underwent surgical treatment without a sure understanding of the nature of lymphoma. Clinical observation showed forms in an advanced state of local development. RESULTS: However, the low specifity of available diagnostic tools and the therapeutic delay are factors that seems do not influence the prognosis, as well as the stage of the disease determined with traditional classification methods. The aspect that seems to be crucial for the prognosis of the disease is the histotype (5 low grade cases free of desease from 36 months to 10 years, vs 4 high grade cases died between 46 days and 40 months after the surgical treatment). CONCLUSIONS: Some interesting considerations arise from the analysis of the diagnostic procedure and from the method of treatment: the specifity and precocity of the diagnosis could be improved by a large use of endoscopic ultrasonography that allow to obtain deep biopsy; the identification of histotype before surgical treatment can address and modulate the therapeutic strategy eliminating the over treatment for L.G. forms.     

Malignant lymphoma of the thyroid gland: a clinical and pathologic study of twenty cases

Among 20 patients with malignant lymphoma of the thyroid gland, the mean age at diagnosis was 63 years and the male to female ratio was 1:6. All patients had a firm, rapidly enlarging neck mass. In 90% of the patients the mass had been present for less than 1 year; in 40%, for less than 1 month. Approximately half of the patients tested had hypothyroidism; three fourths had elevated antithyroid antibodies. There was one nodular lymphoma. The remaining 17 cases available for review were diffuse. Thyroid lobectomy was performed in seven patients, limited excision in eight, and needle biopsy alone in five. External irradiation was administered in 11 cases (55%). Chemotherapy was used alone in one patient (5%) and in combination with radiotherapy in eight (40%). Six patients (30%) were alive without evidence of recurrent disease at follow-up ranging from 1 to 12 years. Eleven patients had died of lymphoma, all but one dying within 1 year. One patient died of other causes and two were lost to follow-up study. There was no appreciable effect of patient age or sex, lymphoma histology, or extent of surgical resection on survival. Treatment of choice for primary lymphoma of the thyroid gland appears to be external irradiation or chemotherapy, alone or in combination. The role of surgery is limited to making a tissue diagnosis of lymphoma, unless the tumor is completely intrathyroidal.     

Primary renal neuroblastoma--a clinical pathologic study of 8 cases

Neuroblastoma is the most common extracranial solid tumor to occur during infancy and early childhood. However, primary renal neuroblastoma is rare, and only scattered case reports exist in the English medical literature. We report 8 cases that accumulated at our institution over the past 15 years and summarize their clinicopathologic features. The composite picture of a patient with renal neuroblastoma is that of a boy of 17 months of age, who presented with a large renal mass, about 9 cm in size, accompanied by hypertension. The mass was typically hemorrhagic, either encapsulated or unencapsuated, and infiltrating. A renal neuroblastoma can be undifferentiated, poorly differentiated, or differentiating; it falls into either the favorable or the unfavorable histology category, and presentation at higher stages is the rule. The N-myc is usually unamplified, and the bone marrow is usually not involved at presentation. Unless the tumor is undifferentiated or very poorly differentiated, patients with renal neuroblastoma fare well, although not without new and improved modalities of treatment. Primary renal neuroblastoma is perhaps more common than people realize; a higher level of awareness and early recognition are important for its prognosis and management, as they are very different from Wilms tumor.     

MALT lymphoma of the gastrointestinal tract: description of clinical cases and review of the literature

MALT-lymphomas are tumors of the mucosa associated lymphoid tissue, without primitive involvement of lympho nodes and spleen. They are located preferentially in the gastroenteric tract specially in the stomach (27%), but they may occur also in the respiratory and urinary tracts. There is a very well known link between MALT-omas and Hp infection, not only in the gastroenteric tract, but also in other sites. Moreover eradication of Helicobacter pylori can, in some cases, contribute to achieve the complete remission of the tumor. In this article the Authors report three cases of MALT-lymphomas: two of the stomach and one of the jejunum-ileum. Diagnosis is based on instrumental tests according to the involved tract and endoscopy of the upper digestive tract associated with biopsies more specific in case of gastric involvement; yet bioptic samples show a more significative incidence of false negative due to the extramucosal site of tumor. In these cases, a correct diagnosis will be obtained only through histologic examination of the surgical specimen. Surgery obtains good results in early stages, playing also a central role in palliation in association with radio- and chemotherapy. In case of low-grade B MALT-lymphomas of the stomach and in IE stages, eradication of Helicobacter pylori can, in 70-90% of cases, reduce the extension of the lymphocytic infiltration.     

Primary gastrointestinal non-Hodgkin's lymphoma: a study of 35 patients

In a retrospective study of 35 patients (29 men, 6 women) with primary non-Hodgkin's lymphomas of the gastrointestinal tract, 13 of the tumours were located in the stomach, 21 in the small bowel and one in the colon. Various radiological findings were detected, and the majority of tumours (23) had high grade histology. Three patients had immunoproliferative small intestinal disease. Only tumours in stages I and II were included in the study, and the majority (25) were in stage IIA. All patients except one had undergone resection of the neoplasm. This was followed by combined chemotherapy in 25 patients, and chemotherapy followed by radiotherapy in three cases. There were two (5.7%) hospital mortalities. Two- and 5-year survival rates were better in those patients with low stage and low grade tumours. The overall 5-year survival rate was 38%.     

Primary cutaneous follicular lymphoma: a clinicopathologic and molecular study of 16 cases in support of a distinct entity

Primary cutaneous B-cell lymphomas displaying a prominent follicular growth pattern are rare and remain poorly defined, particularly in terms of the frequency of detection of t(14;18) and whether or not, as a group, they represent an entity distinct from follicular lymphoma arising in lymph nodes. The morphologic, immunophenotypic, and clinical features of 16 cases of primary cutaneous follicular lymphoma, identified during a review of all PCBCL in the Scotland and Newcastle Lymphoma Group database, were studied and the number of cases harboring t(14;18) assessed by polymerase chain reaction using primers to the major breakpoint cluster region. Comparisons were made with stage I follicular lymphoma arising in lymph nodes and follicular lymphoma secondarily involving the skin. All cases of primary cutaneous follicular lymphoma had undergone thorough staging, including physical examination and CT scans of chest and abdomen, with 15 of 16 cases also having bone marrow aspiration and/or trephine performed. The morphology and immunophenotype of the lesions were similar to that expected in lymph nodes. All cases displayed a follicular architecture complete with follicular dendritic cell networks and comprised an admixture of CD10 and/or bcl-6-positive neoplastic centrocytes and centroblasts with 13 of 16 cases also expressing bcl-2 protein. None harbored t(14;18), a significantly different finding compared with cases of stage I nodal follicular lymphoma (p <0.001) and secondary cutaneous follicular lymphoma (p <0.039). Relapses occurred in five of 15 patients with a median time to first relapse of 20 months (range 1-73 months; mean 27.2 months). These were multiple in two patients and involved extracutaneous sites in two patients. The propensity for relapse was similar to that in a comparative cohort of stage I nodal follicular lymphoma, but the group of primary cutaneous follicular lymphoma were significantly more likely to attain complete remission; all cases of primary cutaneous follicular lymphoma were in complete remission when last seen compared with 49 of 87 patients with stage I nodal follicular lymphoma (p <0.005). No lymphoma-related deaths were encountered in 15 cases with a mean follow-up >60 months (range 5-119 months). These results support the concept of a subtype of follicular lymphoma lacking t(14;18) involving the major breakpoint cluster region, and with a propensity to arise in the skin. Despite a high relapse rate patients with primary cutaneous follicular lymphoma are more likely to achieve complete remission and may ultimately have a more favorable long-term prognosis than those with equivalent nodal disease.     

原发性甲状腺恶性淋巴瘤22例临床分析

目的：总结原发性甲状腺恶性淋巴瘤（PTL）的临床表现、诊断、治疗和预后情况。方法回顾性分析我院2009年至2012年收治的22例PTL患者的临床和病理资料。22例患者均接受手术治疗,术中查8例病变局限于腺体内,14例侵及腺外组织。其中行甲状腺全切1例,双侧次全切除15例,单侧腺叶切除6例,3例同期行中央区淋巴结清扫术。6例患者因术前有气道狭窄在手术同期行气管切开术。结果22例患者术后病理证实为PTL。实验室检查均有甲状腺球蛋白抗体和／或微粒体抗体升高。病理类型为弥漫性大B细胞淋巴瘤（DLBCL型）和黏膜相关淋巴组织淋巴瘤（MALT型）。术后化疗效果良好。最长随访时间61个月。结论 PTL诊断较难,术后病理检查是明确诊断的有效方法。对于迅速增大并伴有压迫症状及抗体增高的甲状腺肿物术前应考虑PTL的可能性,并予以积极处理。甲状腺抗体检查在协助诊断上有一定意义。联合放化疗是临床上治疗的主要方法。两种病理类型淋巴瘤的患者在预后上未见明显差异。     

Primary gastrointestinal malignant lymphoma. An analysis of 63 cases]

Sixty-three patients with primary gastrointestinal malignant lymphoma confirmed by pathology and treated in our hospital from 1973 to 1989 were reported. There were 30 males and 33 females, four suffering hodgkin's diseases and 59 non-hodgkin's lymphoma, with 17 at stage I, 17 at stage II, 9 at stage III, and 20 at stage IV. Patients were treated by surgery, radiotherapy and chemotherapy separately or combined. Forty-seven patients underwent tumor extirpation. Five-year's postoperative survival at stage I, II, and III were 84.6%, 33.3%, and 14.2%, respectively. Surgery combined with postoperative radiotherapy and/or chemotherapy gave better results. The incidence, diagnosis, clinical staging and the therapeutic indications were discussed with a review of literature.     

Hepatoblastoma. A clinical and pathologic study of 54 cases

The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months-4 1/2 years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihypertrophy and Budd-Chiari syndrome (one case), and synchronous Wilms' tumor (one case). Thirty-two of 49 conventional hepatoblastomas were subclassified as epithelial type (19 predominantly embryonal, 13 predominantly fetal), and 12 had mixed epithelial-mesenchymal histology. Pathologic material in five cases was too limited for reliable subclassification. Hepatoblastomas classified as anaplastic type had more primitive histology with a predominance of small- to medium-sized cells in diffuse sheets. The overall mortality was 76% and the average duration of disease from diagnosis to death was 8 months (range: 1 month-27 months). Complete surgical resection remains the key treatment for achieving long-term survival and was attempted in 33 of the 54 children (61%). There were 13 long-term survivors followed for an average of 9 years. The most common type of hepatoblastoma in these children was epithelial with a predominantly fetal pattern. None of the children with anaplastic tumors survived.     

Primary lymphoma of the bladder: a clinicopathological study of 3 cases

Primary involvement of the bladder by lymphoma is rare but is associated with a favourable prognosis if diagnosed early and treated appropriately. The clinicopathological features of 3 cases of primary, non-Hodgkin's lymphoma of the bladder are described. This small study suggests a better prognosis with a favourable histological grade and an uncomplicated lymphoma confined to the bladder. We emphasise the value of aggressive treatment of the tumour.     

Synovial sarcoma of the pleura: a clinical and pathologic study of three cases

Synovial sarcomas are rare soft tissue malignancies that most commonly affect the extremities in the vicinity of large joints. These malignancies typically occur in adolescents and young adults between the ages of 15 and 40 years.(1,2) Historically they are believed to originate from primitive pluripotent mesenchyme capable of synovial differentiation. This belief is consistent with the malignancy's origin from sites devoid of normal synovium, such as the pleural cavity. A variety of pleural cavity sarcomas have been described, including liposarcoma,(3) chondrosarcoma,(4) osteosarcoma,(5) and malignant schwannoma.(6) Pleural synovial sarcoma, however, is a much rarer entity. In fact, pleural synovial sarcoma was first described only 6 years ago(7) and has not yet been reported in the surgical literature. Because of its rarity, pleural synovial sarcoma is often mistaken for the histologically similar malignant mesothelioma, the most common of the pleural neoplasms. This is a critical distinction, because synovial sarcoma may be extremely aggressive. Studies in the last 10 years have shown it to be extremely sensitive to ifosfamide-based chemotherapy, and survival of patients with synovial sarcoma has recently increased with chemotherapy, with 5-year survivals now as high as 57%.(8-10) In this report, we describe 3 cases of synovial sarcoma of the pleura. Clinical findings are correlated with pathologic features, including immunohistochemical stains and fluorescence in situ hybridization (FISH) for the identification of the diagnostic chromosomal translocation, t(X;18)(p11.2;q11.2). This delineation of the clinical and pathologic aspects of this rare, newly recognized tumor should increase awareness among the surgical community.     

Primary lymphoma of bone: a prospective study of 28 cases

PURPOSE: To conduct a prospective study of primary lymphoma of bone (PLB) comparatively with extraskeletal non-Hodgkin's lymphomas (ESNHLs) and secondary lymphoma of bone (SLB). PATIENTS AND METHODS: The 28 cases of PLB, 2932 cases of ESNHL, and 219 cases of SLB included between April 1, 1993, and October 1, 1997, in a treatment protocol for NHL developed by the Adult Lymphoma Study Group, were studied prospectively. RESULTS: Of the 28 PLB patients, 17 were men and 11 women; median age was 48 years (range, 18-69). The disease was monostotic in 17 cases (involving the peripheral skeleton in 14) and polyostotic in nine cases. The proportion of patients younger than 60 years was 86% in the PLB group, 59% in the ESNHL group, and 55% in the SLB group. The Ann Arbor stage distribution (I-II/III-IV) was as follows: 54%/46% in the PLB group, 50%/50% in the ESNHL group, and 20%/80% in the SLB group. Performance status was 0 or 1 in 100% of the PLB patients, 50% of the ESNHL patients, and 20% of the SLB patients. The phenotype was B in 89% of the PLBs and 85% of the ESNHLs and SLBs. In the PLB group, 54% of patients had diffuse large cell tumors and 11% diffuse mixed tumors; in the ESNHL group, 39% had diffuse large cell, 13% diffuse mixed, and 8% diffuse immunoblastic tumors; and in the SLB group, 45% had diffuse large cell, 10% diffuse mixed, and 12% diffuse immunoblastic tumors. A complete or partial response to induction therapy was noted in 86% of PLB patients, 84% of ESNHL patients, and 78% of SLB patients. Overall five-year survival was 65% in the PLB group, 50% in the ESNHL group, and 40% in the SLB group. DISCUSSION: Survival was better in the PLB group. Further studies are needed to determine the effect of radiation therapy at completion of the treatment protocol and to look for prognostic factors associated with bone involvement.     

23例脾脏原发性恶性淋巴瘤临床分析

目的 提高临床对少见的脾原发性恶性淋巴瘤诊断和治疗的水平。方法 对1956年1月至1999年8月收治的23例脾原发性恶性淋巴瘤临床资料进行回顾性分析。结果 23例均行手术探查,22例获切除,术后均行COP或CHOP方案化疗。23例均为术后病理确诊,病理类型均为NHL,其中B细胞源性21例,T细胞源性2例。按Ahman脾恶性淋巴瘤临床分期,I期9例、Ⅱ期8例、Ⅲ期6例,5年生存率分别为50％、40％、16％。结论 脾恶性淋巴瘤的诊断主要依靠B超和CT,病期的早晚影响其预后,以手术为主的综合治疗是本病的主要治疗方法。