Mediastinal masses in children

A variety of mediastinal masses may present in children. Imaging is generally confined to chest radiograph and CAT scan of the chest, although other studies may prove helpful depending upon the circumstances. MRI is particularly helpful for neurogenic tumors in the posterior mediastinum. The most common tumor in the anterior mediastinum is lymphoma with germ cell tumors second. In general, lymph nodes in areas outside the mediastinum provide access for tissue diagnosis when lymphoma is present. Germ cell tumors are generally benign, but surgical excision is indicated to rule out malignant elements and to treat symptoms which are often present through compression of nearby structures. Neurogenic tumors are the most common lesions in the posterior mediastinum.     

Surgical approach to isolated mediastinal lymphoma

With the aim of assessing the role of surgery in the management of isolated mediastinal lymphoma, we have reviewed the data of 123 operations performed on 102 patients (64 with Hodgkin's disease and 38 with non-Hodgkin's lymphoma). One death and four major complications occurred in these patients. Macroscopically radical resection was performed in 14 patients who are free of disease after 1 to 14 years. Debulking resection was performed in five patients: Three are alive after 5 to 11 years and two died after 36 and 40 months. Ten patients (seven with non-Hodgkin's lymphoma and three with Hodgkin's disease) had residual mediastinal masses of more than 2 cm after chemotherapy; to assess the nature of the lesion (fibrosis or residual disease), we subjected these patients to surgical restaging of the mediastinum: Results were negative in seven and positive in three. We conclude that open biopsy is indispensable to obtain good tissue specimens suitable for histologic and immunohistochemical assessment. Biopsy must be performed as a major surgical procedure to avoid reoperation: Mediastinoscopy and sternal splitting incisions proved the most reliable approaches. Locally radical or debulking resection might be considered in selected cases to enhance long-term results.     

Bone marrow involvement in lymphomas with hemophagocytic syndrome at presentation: a clinicopathologic study of 11 patients in a Western institution.

Hemophagocytic syndrome (HPS) is a clinicopathologic syndrome that can reveal a non-Hodgkin's lymphoma. The pathologic features of lymphoma associated with HPS remain ill defined. We studied 11 lymphomas associated with HPS on initial bone marrow biopsies, consecutively diagnosed during a 6-year period in a Western institution. There were seven diffuse large B-cell lymphomas (DLBCLs), three T-cell lymphomas (one peripheral T-cell lymphoma unspecified, two hepatosplenic gammadelta T-cell lymphomas [HS gammadeltaTLs]), and one aggressive NK-cell lymphoma/leukemia (NKL). These lymphomas shared common clinicopathologic features with a systemic presentation, a poor outcome (nine patients died within 2 years), and a mild interstitial lymphoid infiltrate of the bone marrow at presentation in nine patients. This equivocal lymphoma infiltrate was blending with normal hematopoietic cells, and CD20 and CD3 immunolabelings were essential for its detection. A high number of reactive T (CD3+) cells, most often with a predominant cytotoxic (CD8+ TiA1+) phenotype, was present in all DLBCLs. By in situ hybridization, Epstein-Barr virus was detected in neoplastic cells of three cases (one DLBCL, one HS gammadeltaTL, and one NKL), which also showed serum viral DNA. Polymerase chain reaction studies disclosed HHV6 DNA sequences in tumor tissues of two DLBCLs, whereas HHV8 DNA was not detected. Because tumor mass indicative of lymphoma was not striking in most patients, bone marrow biopsy appears to be of great value for the diagnosis of an HPS-associated lymphoma, which may be, in Western patients, of B- as well as T- or NK-cell type. Immunostaining for CD3 and CD20 is essential to identify the common subtle lymphoma involvement. Together with a better understanding of the pathogenic processes, an early diagnosis may improve the prognosis of HPS-associated lymphoma.     

Videothoracoscopy for the management of mediastinal mass lesions

Background: The indications for video-assisted thoracoscopy have steadily expanded during recent years and include now the management of various mediastinal disorders. Methods: Until now we have used videothoracoscopy for the diagnosis or treatment of mediastinal mass lesions in 28 patients. The indication for the procedure was bilateral or unilateral mediastinal adenopathy in 16, a suspected malignant anterior mediastinal mass lesion in six, and a presumable benign tumor of the posterior or anterior mediastinum in six patients. Results: Video-assisted thoracoscopy provided an accurate tissue diagnosis in all patients with adenopathy and in all but one patient with a malignant mass lesion of the anterior mediastinum. It further allowed complete excision of all benign tumors of the anterior or posterior mediastinum. There were no intra- or postoperative complications, but conversion to open thoracotomy was necessary in one patient. Conclusions: Video-assisted thoracoscopy is a valuable adjunct to traditional surgical techniques for the diagnosis of malignant mediastinal disease and may overcome some of the limitations of mediastinoscopy and mediastinotomy. In the future, it may become the procedure of choice for the resection of small benign tumors of the anterior or posterior mediastinum.     

Parotid lymphomas--clinical and computed tomographic imaging features

OBJECTIVE: To review the clinical presentation and computed tomography (CT) imaging characteristics of all parotid lymphomas diagnosed at the study institution over a 7-year period. DESIGN: Retrospective chart review of parotid lymphomas diagnosed between 1997 and 2004. SUBJECTS: A total of 121 patients with parotid lesions were identified. After retrospective chart review, a total of 10 patients with histologically proven parotid lymphoma were included in the study, 8 of whom had CT scans available for assessment. RESULTS: Ten patients with histologically proven lymphoma of the parotid gland were identified from among 121 patients with parotid neoplasms, an incidence in this series of 8.3%. All lymphomas were of non-Hodgkin's type. All patients presented with a painless unilateral parotid swelling. Most patients had a short history of less than 4 months' duration, of whom 3 presented with a rapidly evolving swelling of less then 1 month's duration. No patient had a background of Sj?gren's disease or any other autoimmune disorders. The commonest finding noted on CT was of a unilateral, single mass of relative soft-tissue homogeneity with poorly defined, indistinct tumour margins. Associated loco-regional lymphadenopathy was identified in 2 cases, 1 clinically and another radiologically; multiple ipsilateral lesions were noted in 2 cases. No cases of contralateral disease were observed. CONCLUSION: Lymphoma has a clinical presentation similar to other neoplasms arising within the parotid gland. A unilateral, non-tender swelling was a universal finding. A history of less than 4 months may suggest the possibility of lymphoma. CT scanning is a useful adjunctive investigation to determine the site and extent of the disease, loco-regional nodal status and contralateral gland and neck status. Multifocality and associated adenopathy are associated with, but not exclusive to, parotid lymphoma. Although poor tumour boundary definition on CT imaging is a strong predictor of malignancy, no pathognomonic finding specific for lymphoma has been identified. The potential diagnosis of parotid lymphoma should be considered in all patients who present with a parotid mass.     

A current analysis of primary lymphoma of the thyroid

Over a 9-year period ending in May 1990, 27 patients with histologically proved thyroid lymphoma were assessed and treated. There were 24 female and three male patients with a median age of 67 years at the time of diagnosis (age range, 39 to 85 years). The usual presentation was that of a rapidly enlarging neck mass. Incisional biopsy was the diagnostic procedure of choice; however, nine of 27 patients underwent diagnostic partial or total thyroidectomy based on a preoperative impression of thyroid carcinoma. All 27 patients had non-Hodgkin's lymphomas of intermediate (77%) or high (23%) grade. Detailed staging was carried out in 25 patients; seven patients (28%) had disease confined to the thyroid gland (stage I), while 18 (73%) had accompanying disease in cervical lymph nodes or the mediastinum (stage II). Combined multiagent chemotherapy and irradiation was given to 19 of 25 staged patients (76%). Actuarial, overall 5-year survival for all patients was 70% with 48 months being the median follow-up for living patients (follow-up range, 3 to 102 months). Of a number of factors evaluated using log-rank survival tests, only the absence of dysphagia at the time of hospital admission, a primary tumor mass not greater than 10 cm, restriction of disease to the thyroid gland, and the absence of mediastinal lymph node involvement were statistically significant predictors of improved survival. Surgery should usually be restricted to diagnostic biopsy, as there is infrequently a role for resection in the management of thyroid lymphoma, given the effectiveness of combined multiagent chemotherapy and radiotherapy.     

Primary colorectal lymphomas

OBJECTIVE: The incidence of primary colorectal lymphomas is rare, comprising 10-20% of gastrointestinal lymphomas and only 0.2-0.6% of large bowel malignancies. There is a male predominance, with a maximal reported incidence in the 50- to 70-year age group. Patients often present delayed with nonspecific symptoms and consequently have advanced disease at the time of diagnosis. Inflammatory bowel disease and immunosuppression have been reported as risk factors, although a direct causal link has yet to be established. Treatment often involves a multimodality approach, combining surgery and chemotherapy, with the use of radiotherapy in selected cases. We present our experience in the management of primary colorectal lymphomas over a 10-year period (1989-1999). PATIENTS AND METHODS: We reviewed all cases of primary colorectal lymphoma seen at our institution from 1989 to 1999. Patients were included based on standard diagnostic criteria for primary intestinal lymphoma established by Dawson in 1961. The following clinical information was obtained: age, sex, presentation, site of tumour, operation performed, histology, length of stay, intraoperative complications, adjuvant therapy and duration of follow-up. The type of lymphoma was classified according to the WHO classification system. For staging, a modification of the Ann Arbor system for gastrointestinal lymphoma, proposed by Musshoff, was used. RESULTS: During the 10-year period from 1989 to 1999, 14 cases of primary colorectal lymphomas were identified. This comprised 0.44% of all colorectal malignancies (14 of 3199 cases) seen in our department during this period. There were 13 men and one woman. Their mean age at presentation was 61 years. The common presentations included a combination of abdominal pain (71.4%), anorexia and loss of weight (42.9%) and an abdominal mass (28.6%). The two most common sites of involvement were the caecum (57.1%) and the rectum/sigmoid colon (21.4%). The lesions manifested in a variety of ways, ranging from solitary fungating masses to multiple colonic polyps. All but one patient underwent attempted curative surgical resection. All cases were non-Hodgkin's B-cell lymphomas, with a majority being diffuse large B-cell lymphomas (57.1%). The diagnoses were established through laparotomy in all cases. All cases presented with evidence of spread to regional lymph nodes or beyond. Eleven patients (78.6%) received postoperative chemotherapy, with a regimen that included cyclophosphamide, vincristine, doxorubicin and prednisone. The remaining three were too ill or refused. There were no mortalities within 30 days of surgery. The patients were followed up for a median of 20 months (range 2-84) and more than half are alive and continue to be on follow-up with no evidence of recurrence. CONCLUSION: Primary colorectal lymphoma is a rare condition. It predominantly affects males between the sixth and seventh decade of life and most commonly occurs in the caecum. It often presents with abdominal pain and loss of weight and due to the nonspecific nature of these symptoms, patients frequently present late with advanced loco-regional disease. The histology is usually B cell and of intermediate grade. Therapy usually involves resection of the affected colon and regional lymphovascular structures, followed by adjuvant chemotherapy, with a reported 5-year survival of 27-55%.     

High specificity of combined TRAP and DBA.44 expression for hairy cell leukemia

Because of marrow fibrosis, bone marrow aspirations are often nonconclusive in patients with hairy cell leukemia (HCL). Therefore, histologic examination is important in HCL but often difficult in cases with low numbers of tumor cells. A combined immunohistochemical positivity for DBA.44 and tartrate-resistant phosphatase was previously found in 100% of HCL and suggested to be specific for this diagnosis. To further assess the diagnostic specificity and sensitivity of this immunohistochemical approach in a higher number of cases, we analyzed 56 HCLs and lymphoma tissue microarrays, including 840 cases of the most frequent non-Hodgkin lymphomas. All HCLs showed combined positivity for these two proteins (100% sensitivity). Both antibodies were often positive in other lymphoma types. DBA.44 reactivity was especially frequent in follicular lymphomas (46%), whereas tartrate-resistant acid phosphatase (TRAP) expression was often seen in mantle cell lymphomas (57%), primary mediastinal large B-cell lymphomas (54%), and chronic lymphocytic leukemia/small lymphocytic lymphoma (41%). A combined DBA.44/TRAP positivity was seen in only 3% of non-HCL non-Hodgkin lymphomas, including cases of diffuse large B-cell lymphomas, follicular lymphomas, chronic lymphatic leukemia/small lymphocytic leukemias, and mantle cell lymphomas. Overall, these data confirm the utility of combined immunohistochemical DBA.44/TRAP expression analysis in confirming the diagnosis of HCL. However, combined positivity for these markers is highly sensitive but not absolutely specific for HCL.     

Acquired immunodeficiency syndrome-associated malignancies

Acquired immunodeficiency syndrome (AIDS)-associated malignancies are a concern to all physicians. Because patients usually are seen initially by nonspecialists, primary care physicians, especially, may find useful information about AIDS-related malignancy. Cancers remain a part of the clinical definition of AIDS. Kaposi's sarcoma (KS) and non-Hodgkin's lymphomas are the malignancies most frequently seen in AIDS. Acquired immunodeficiency syndrome KS occurs in young men between 20 and 40 years of age, and involves the skin in more than 90% of patients. Visceral dissemination is common. The prognosis is related to immune functional status and the extent of disease involvement. Acquired immunodeficiency syndrome-related non-Hodgkin's lymphoma is generally a B-cell malignancy. Advanced disease at presentation and proclivity for central nervous system involvement are characteristic. Knowledge of the common manifestations of these cancers, methods of diagnosis, and potential treatment options is important for physicians.     

Anterior mediastinal presentation of a giant angiomyolipoma

Angiomyolipomas are benign, solitary, noninvasive lesions that most often arise in the kidney. Extrarenal manifestations of these tumors include the skin, oropharynx, the abdominal wall, retroperitoneum, gastrointestinal tract, heart, lung, liver, uterus, penis, and spinal cord. We report a patient with a giant angiomyolipoma located in the anterior mediastinum. We believe this is the seventh reported case of mediastinal angiomyolipoma and the largest reported by size. It is the second reported lesion to arise in the anterior mediastinum. Distinction from other pulmonary or thoracic masses relies on the appreciation of the unique and characteristic histologic features of these mediastinal angiomyolipomas. We conclude that, although rare, angiomyolipoma should be considered in the differential diagnosis of a mediastinal tumor.     

Follicular lymphoma of the thyroid gland

The majority of lymphomas arising in the thyroid gland are mucosa-associated lymphoid tissue lymphomas and diffuse large B-cell lymphomas, which arise from a background of chronic lymphocytic thyroiditis. Follicular lymphoma may also present in the thyroid gland, but its clinicopathologic features at this site are not well characterized, leading to difficulties in diagnosis and clinical management. We have addressed this problem by studying the clinical, morphologic, immunophenotypic, and genetic features of 22 such cases. All cases showed morphology characteristic of follicular lymphoma, however, in many the interfollicular neoplastic infiltrate was particularly prominent and all lymphomas contained readily identifiable and often striking lymphoepithelial lesions, features heretofore considered indicative of mucosa-associated lymphoid tissue lymphoma at this site. Furthermore, 13 of 18 cases for which sufficient evidence was available had clinical and/or histologic evidence of chronic lymphocytic thyroiditis. Analysis of genetic and immunohistochemical features identified 2 distinct groups. In 1 group, similar to typical adult follicular lymphoma, cases carried a t(14;18)/IGH-BCL2 and/or expressed Bcl-2, and were mostly CD10-positive and of World Health Organization (WHO) grade 1 to 2. Follicular lymphomas in the other group lacked IGH-BCL2 and Bcl-2 expression, were often of WHO grade 3 and were often CD10-negative, similar to the minority of follicular lymphomas previously described that are Bcl-2-negative and are often encountered at other extranodal sites. The 2 groups differed in clinical stage at presentation, 11 patients in the former group but none in the latter group having disease beyond the thyroid gland. Appreciation of the spectrum of morphologic, immunophenotypic, and genetic characteristics of follicular lymphoma presenting in the thyroid gland should aid both diagnosis and clinical management.     

Primary Lung Small B-Cell Lymphoma versus Lymphoid Hyperplasia: Evaluation of Diagnostic Criteria in 26 Cases.

Primary lung non-Hodgkin's lymphoma is a rare neoplasm mostly represented by low-grade B-cell lymphomas of mucosa-associated lymphoid tissue. Their diagnostic criteria are now well defined on surgical specimens, but pathologists may experience difficulties in distinguishing them on exiguous biopsies from benign lymphoid hyperplasia and other lymphomas. Therefore, we examined a series of 26 lung lymphoid lesions to further define the pathologic features of either lymphoma or lymphoid hyperplasia on small specimens. We observed 16 primary lung non-Hodgkin's lymphomas with a large predominance of low-grade mucosa-associated lymphoid tissue-type lymphomas (87.5%, n = 14). There were no autoimmune disorders, but three patients had a concomitant infectious disease (hepatitis C virus and Helicobacter pylori gastritis). One patient presented with a synchronous pulmonary adenocarcinoma. As well as the classical mucosa-associated lymphoid tissue cellular infiltrate, immunohistochemical characterization of the 14 mucosa-associated lymphoid tissue-type lymphomas revealed the CD20+/CD43+ centrocyte-like cell phenotype in 10 cases (71.5%). Although the lymphoepithelial lesions observed in all lymphomatous cases have been reported in lung lymphoid hyperplasia, the determination of B-cell CD20+/CD43+ phenotype of the intraepithelial lymphocytes highly increased the specificity of lymphoepithelial lesions. A monoclonal immunoglobulin heavy chain gene rearrangement was present in 71.4% of the mucosa-associated lymphoid tissue-type lymphoma specimens. Investigation of H. pylori by polymerase chain reaction detection was negative, even for the two cases associated with H. pylori gastritis.     

纵隔镜在仅以纵隔占位为表现的疾病诊断中的价值

目的 探讨纵隔镜在仅以纵隔占位为表现的胸部疾病的诊断中的价值,分析其临床表现与病理诊断的关系.方法 2000年7月至2006年3月97例诊断不明的仅以纵隔占位为表现的胸部疾病病人行纵隔镜检查,其中行颈部纵隔镜69例,胸骨旁纵隔镜28例.结果 手术过程均顺利,无严重手术并发症及围手术期死亡.除1例外,96例获得明确病理诊断.尽管病人临床症状无特异,但若伴有声音嘶哑、吞咽困难、下肢水肿、上腔静脉阻塞综合征等症状,则恶性肿瘤的可能性极大.前上纵隔病变以淋巴瘤（7/18例）、胸腺肿物（4/18例）、小细胞癌（4/18例）为主;中纵隔的病变以转移性非小细胞肺癌（11/37例）、结节病（9/37例）、小细胞癌（7/37例）和淋巴结反应性增生（6/37例）为主;合并肺门淋巴结肿大的纵隔病变以结节病（32/41例）,小细胞癌（5/41例）和结核（3/41例）为主.结论 经纵隔镜行纵隔病变活检为仅以纵隔占位为表现的胸部疾病病人提供了安全精确的诊断方式,从一些特征性临床表现中可以获得对肿物性质的初步判断.     

Atypically located pericardial cysts

Pericardial cysts are uncommon benign abnormalities and are most often found in either cardiophrenic angle. We present the cases of 3 patients with a cyst in the mediastinum and review the literature. Pathological examination confirmed the diagnosis of pericardial cyst. Clinicians should include pericardial cyst in the differential diagnosis of mediastinal masses.     

Spontaneous pneumomediastinum: A report of five cases

Conclusion SPM is a self-limiting disease in which symptoms subside with conservative management in most cases. However, drainage of mediastinal air may be inevitable when vital structures of the mediastinum are severely compromised. In the presence of symptoms which are highly suggestive of SPM, obtaining a CT of the chest will confirm the diagnosis. If not suspected clinically, more invasive diagnostic tools such as oesophagoscopy and/or bronchoscopy may not be warranted in order to rule out perforation of an air containing mediastinal organ.     

Rare oral cavity presentation of a B-cell lymphoblastic lymphoma. A case report and review of the literature.

Lymphoblastic lymphoma is an uncommon malignancy, with most cases showing a T-cell phenotype and presenting as a mediastinal mass. By contrast, B-cell lymphoblastic lymphoma/leukemia is a rare high-grade malignancy that comprises approximately 10% of all lymphoblastic lymphomas. Lymphomas of the oral cavity are rare and typically present as intraosseous lesions that are most commonly diffuse large B-cell type. Here we present what we believe is the first B-cell lymphoblastic lymphoma initially presenting in the oral cavity. The case involves a 46-year-old white woman who presented with a mass in the right mandible. This report discusses this rare malignancy, including clinical presentation, histopathologic features, immunologic profile, treatment, and prognosis. This case emphasizes the importance of recognizing rare entities that may present in the oral cavity and the impact of the disease and its management.     

Florid epithelioid cell and sarcoid-type reaction associated with non-Hodgkin's lymphoma

Five patients suffering from non-Hodgkin's lymphoma associated with an intense infiltration of epithelioid histiocytes and even sarcoid-type granulomas in the involved tissue are described. A similar tissue reaction, at both involved and uninvolved sites, has been described in patients with Hodgkin's disease. The cause of this reaction may be immunological; its prognostic significance still awaits elucidation. In the case of non-Hodgkin's lymphomas, it may cause difficulty in diagnosis of lymphoma and hence may result in unnecessary delay in the initiation of therapy.     

Preoperative distinction of parotid lymphomas

BACKGROUND: Surgical parotidectomies place parotid lymphoma patients at increased risk of morbidity because of the diffuse infiltrative nature of the disease. These tumors usually respond to radiotherapy or chemotherapy, but are difficult to distinguish from other benign parotid gland tumors preoperatively. We sought to identify clinical and radiologic features that could aid in the preoperative distinction of parotid lymphomas. STUDY DESIGN: We performed a retrospective chart review of 248 superficial parotidectomy patients. RESULTS: Of 248 parotid lesions, 22 (8.8%) were lymphomas (21 non-Hodgkin's, 1 Hodgkin's). The lymphoma cases included four patients with a history of autoimmune disease, and four with a previous diagnosis of lymphoma. Palpable painless masses were present in all cases (14 right, 7 left, 1 bilateral). Two patients had multiple palpable parotid nodules and five had palpable cervical adenopathy. Of 17 patients receiving preoperative CT scans, 4 more patients were revealed to have bilateral disease, 7 more patients had cervical adenopathy, and 5 more patients had multiple parotid nodules. Tumors were poorly circumscribed in 7 of 17 (41%), and diffuse parotid changes were noted in 2. Preoperative fine needle aspiration biopsy was performed in 10 of the 22 lymphomas, revealing 3 reactive nodes, 3 suspicious for lymphoma and 3 nondiagnostic. CONCLUSIONS: Although parotid lymphomas are uncommon, a history of autoimmune disease or previous lymphoma; clinical or radiologic evidence of bilateral, multiple, or poorly circumscribed parotid lesions; and cervical lymphadenopathy should suggest this diagnosis. This may alter the proposed surgery and decrease the potential morbidity associated with superficial parotidectomy in cases of parotid lymphoma.     

Histoplasmosis simulating lymphoma in children.

The experience of two Mineapolis hospitals with histoplasmosis over a 10-yr period was reviewed. Six of eleven pediatric patients with histoplasmosis presented with systemic symptoms, a mediastinal mass, and no pulmonary infiltrates. A clinical diagnosis of lymphoma was made in each case. Immediate radiation therapy for respiratory compromise was considered in one patient before pathologic diagnosis revealed primary histoplasmosis of the mediastinum. Although immediate therapy with steroids or irradiation is often considered in patients who have acute respiratory embarrassment caused by a mass, histologic confirmation of the diagnosis is always indicated before such therapy is instituted. Primary histoplasmosis of the mediastinum may be more common than previously thought.     

Benign teratomas of the mediastinum

Approximately 8% of all mediastinal tumors are benign teratomas. We reviewed 86 cases of benign teratoma seen at the Mayo Clinic from 1930 through 1981. The mean age of the patients was 28 years and the sex distribution was approximately equal. The most common symptoms were chest, back, or shoulder pain, dyspnea, and cough, but 36% were asymptomatic at the time of presentation. Chest roentgenograms showed a well-circumscribed anterior mediastinal mass which often protruded into one lung field. Detectable calcification was observed in 22 patients: a calcified tumor wall in seven, bone or teeth in the mediastinum of seven, and nonspecific calcifications in eight. Surgical excision remains the best means of diagnosing and treating this benign tumor. Though the tumors are histologically benign, they may present difficult surgical problems because of the vital structures involved. Since 1952 there has been a change in the clinical presentation of patients with this entity: More patients are asymptomatic and have smaller tumors and fewer complications than prior to 1952.