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J A Ferry

Publications and source records attributed to J A Ferry.

At least 37 records · Page 2Linked to original sources
PubMed1997

Granulocytic sarcoma of the testis: a report of two cases of a neoplasm prone to misinterpretation.

We report the cases of two men, aged 48 and 71 years, with granulocytic sarcoma of the testis. Both presented with left testicular swelling and underwent orchiectomy, which revealed cream-colored to yellow-tan, rubbery-to-firm, testicular tumors with extensive paratesticular spread. The tumor in the younger patient was composed of a uniform population of primitive cells with scant cytoplasm and was initially misinterpreted as malignant lymphoma. Staging revealed no extrascrotal spread. The patient was treated with radiation and chemotherapy and remained free of disease for 12 years, at which time he died of unrelated causes. The older patient had a history of a myelodysplastic syndrome. His tumor contained cells with bright eosinophilic, occasionally granular cytoplasm, consistent with myeloid lineage. Because of a prominent component of myelocytes, with round, eccentric nuclei and moderately abundant cytoplasm, and because of an associated chronic inflammatory cell infiltrate that contained mature plasma cells, the tumor was initially misinterpreted as a plasmacytoma, although it was reinterpreted as a granulocytic sarcoma before initiation of therapy. Tumor cells in both cases were positive with a chloroacetate esterase stain. Immunohistochemical staining revealed expression of myeloperoxidase, lysozyme, leukocyte common antigen, and CD43, but not of B-cell-specific or T-cell-specific antigens in both cases. Granulocytic sarcomas are apt to be misinterpreted as other hematolymphoid tumors, which may result in a significant error in management. The diagnosis should at least be thought of any time the diagnosis of malignant lymphoma or plasmacytoma of the testis is being considered.

Aged↗
PubMed1996

CD5+ extranodal marginal zone B-cell (MALT) lymphoma. A low grade neoplasm with a propensity for bone marrow involvement and relapse.

Three cases of extranodal marginal zone B-cell lymphoma (low grade B-cell lymphoma of mucosa-associated lymphoid tissue [MALT] type) in which the neoplastic B cells expressed the CD5 antigen are reported. The patients included 2 men and 1 woman, aged 44, 62, and 77 years. In all three cases, the histologic features were typical of marginal zone/MALT lymphoma, with reactive follicles, marginal zone (centrocyte-like) cells, and plasma cells. Pseudofollicles, prolymphocytes, and paraimmunoblasts were absent. In all cases, lymphoma from one or more sites expressed monotypic immunoglobulin (2 IgM kappa, 1 IgM lambda), pan B cell antigens and CD5. Two of 3 cases expressed CD43; one case expressed CD23. No case showed overexpression of the bcl-1 protein, cyclin D1. Interphase cytogenetic analysis revealed trisomy 3 in one of two cases examined. The two male patients presented with lymphoma in the ocular adnexa. One of them had marrow involvement, cervical lymphadenopathy and peripheral blood involvement at presentation; 24 months later, he developed a relapse in subcutaneous tissue. The second patient had marrow involvement 3 years later, at the time of recurrence of his orbital disease. The third patient presented with lymphoma at the base of the tongue. She subsequently developed lymphoma involving the left upper eyelid and right lacrimal sac and duct, the marrow, and the nasopharynx between 63 and 95 months after initial presentation. All of these patients presented with disease involving sites in the head and neck and all had multiple relapses or recurrences with bone marrow involvement at the time of presentation (1 case) or at relapse (2 cases). The presence of CD5 may be a marker for cases of MALT lymphoma with a tendency for persistent or recurrent disease, for dissemination to the marrow and other extranodal sites, and for leukemic involvement of the peripheral blood.

Adult↗
PubMed1996

Marginal zone lymphoma (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type) of skin and subcutaneous tissue: a study of 15 patients.

Extranodal low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type occurs in the gastrointestinal tract, salivary gland, thyroid, orbit, lung, and breast. We report 15 patients with MALT-type lymphomas involving skin and subcutaneous tissue. All patients had tumors with histologic features of low-grade B-cell lymphoma of MALT type, including marginal zone cells (15 of 15 cases), plasmacytic differentiation (10 of 15 cases), Dutcher bodies (three of 15 cases), and reactive germinal centers (10 of 15 cases). All expressed pan B-cell antigens and monotypic immunoglobulin. Seven patients (five women, two men) aged 29 to 86 years (median, 53 years) had primary MALT-type lymphoma of skin (6) or subcutaneous tissue (1). One patient had persistent disease, and four patients had relapses involving skin, subcutaneous tissue, breast, orbit, and lymph node. At last follow-up (11-121 months; median, 36 months), one patient was alive with disease, and six patients had no evidence of disease. Three patients (two women, one man) aged 36 to 67 years (median, 57 years) had concurrent MALT-type lymphoma involving both subcutaneous tissue and extracutaneous sites at primary diagnosis, including lung, breast, orbit, lymph node, and bone marrow. One patient responded to treatment but relapsed with lymphoma of the skin and breast. The other two patients had persistent disease despite treatment. One patient died of disease at 25 months, and, at last follow-up (7 and 46 months), two patients were alive with disease. Five patients (four women and one man) aged 29 to 72 years (median, 63 years) had secondary skin or subcutaneous involvement by MALT-type lymphoma with primary tumors of ocular adnexa (3) or parotid gland (2). All five patients had relapses, which involved skin or subcutaneous tissue, parotid gland, lacrimal gland, breast, and lymph node. At last follow-up (61-137 months), two patients were alive with disease and three were alive with no evidence of disease. Low-grade B-cell lymphomas of MALT type may arise in or secondarily involve the skin and subcutaneous tissue and have a tendency to affect middle-aged to older women. These tumors are characterized by multiple extranodal relapses and are associated with long patient survival. Patients with primary MALT-type lymphoma of skin or subcutaneous tissue without extracutaneous involvement at diagnosis were more likely to experience prolonged disease-free survival than patients with extracutaneous spread at presentation (p < 0.03).

Adult↗
PubMed1996

The management and outcome of stage IAE nonHodgkin's lymphoma of the testis.

PURPOSE: The initial management of stage I nonHodgkin's lymphoma of the testis is by orchiectomy but the role and efficacy of adjuvant strategies are uncertain. We reviewed cases of lymphoma at our institution to determine whether adjuvant treatment was beneficial. MATERIALS AND METHODS: A retrospective review of outcome was conducted on 26 patients who presented to our institution. Median followup for the group was 54 months. Kaplan-Meier actuarial analyses were performed on the entire group and subsets. RESULTS; Actuarial 5 and 10-year overall survival rates were 79% and 63% and relapse-free survival rates were 61% and 46%, respectively. In patients who received adjuvant combination chemotherapy the 5-year relapse-free survival rate improved (75% versus 50%) but effect did not achieve statistical significance and was lost by 10 years. No relapse-free survival advantage was noted for patients receiving adjuvant irradiation to the pelvic and para-aortic nodes. Patients who did not receive irradiation remained free of isolated relapses in the pelvic or para-aortic regions. CONCLUSIONS: These data lend support to the use of adjuvant chemotherapy but do not support a role for adjuvant nodal irradiation.

Actuarial Analysis↗
PubMed1995

Uterine papillary serous carcinoma (UPSC): a clinicopathologic study of 30 cases.

Between 1975 and 1989, 896 patients were treated for endometrial carcinoma at the Massachusetts General Hospital. Thirty patients were identified from the tumor registry as having uterine papillary serous carcinomas. The survival for all patients and for groups of patients stratified on clinical and pathological parameters was examined in the Kaplan-Meier survival curves. Curves for the different strata were compared using the logrank test. The 5-year survival for the 30 patients was 30% +/- 9%. Patients with surgical stage I and II tumors had a 5-year survival rate of 79% +/- 14% compared to 25% +/- 10% in patients with stage III and IV tumors (P = 0.02). Clinical stage, depth of myometrial invasion, lymph-vascular space invasion, tumor grade, and DNA aneuploidy were not found to significantly impact on survival. However, a survival advantage was seen in patients diagnosed with early surgical stage tumors, reinforcing the need for thorough staging at the time of laparotomy.

Aged↗
PubMed1995

Interleukin-2 receptor alpha chain regulates the size and content of the peripheral lymphoid compartment.

Interleukin-2 receptor alpha chain (IL-2R alpha) expression occurs at specific stages of early T and B lymphocyte development and is induced upon activation of mature lymphocytes. Young mice that lack IL-2R alpha have phenotypically normal development of T and B cells. However, as adults, these mice develop massive enlargement of peripheral lymphoid organs associated with polyclonal T and B cell expansion, which, for T cells, is correlated with impaired activation-induced cell death in vivo. Older IL-2R alpha-deficient mice also develop autoimmune disorders, including hemolytic anemia and inflammatory bowel disease. Thus, IL-2R alpha is essential for regulation of both the size and content of the peripheral lymphoid compartment, probably by influencing the balance between clonal expansion and cell death following lymphocyte activation.

Animals↗
PubMed1995

Ocular adnexal lymphoma. A clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type.

PURPOSE: Extranodal marginal zone B-cell lymphoma (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue [MALT] type) is a distinctive type of lymphoma that usually arises in association with mucosa or other epithelial structures and has an indolent clinical course. The frequency and clinical features of MALT lymphomas in the ocular adnexa have not been well studied. METHODS: The authors examined the clinicopathologic features of ocular adnexal lymphoma, identified a subset of cases with MALT characteristics, and determined patient outcome. RESULTS: The 42 patients, 16 men and 26 women age 35-89 years (mean, 64) were followed an average of 4.8 years. Thirty-two patients had ocular adnexal involvement at presentation (primary ocular adnexal lymphoma) and 10 had a history of lymphoma that relapsed in the orbit (secondary ocular adnexal lymphoma). In the primary group, 23 patients had lymphoma confined to the ocular adnexa, 3 had a single lesion that invaded adjacent structures, and 6 had distant spread at the time of presentation. Twenty-five patients achieved a complete remission. Nine patients, including 6 patients whose disease was localized initially, had progression or relapse of disease in distant sites. At last follow-up, 21 patients were free of disease, 9 were alive with disease and 2 had died of lymphoma. In the secondary group, at last follow-up, 1 patient had died of other causes, free of lymphoma, 3 patients were alive with disease and 5 had died of lymphoma (outcome not known in 1 case). Using the recently described revised European-American lymphoma classification, we found 16 MALT lymphomas, 8 diffuse large B cell, 12 follicular center, 3 mantle cell, 1 B-small lymphocytic lymphoma, and 2 unclassifiable low-grade lymphomas. The most common type of primary lymphoma was MALT type (15 of 30 classifiable cases), and the most common secondary lymphoma was follicular center (6 of 10). No increased frequency of conjunctival or lacrimal gland involvement by MALT lymphomas was found. All 33 lymphomas with immunophenotyping were of B lineage. CONCLUSIONS: Ocular adnexal lymphomas are B-cell tumors that develop in older adults, predominantly among women. Primary orbital lymphomas have a favorable prognosis; a high proportion of them have MALT characteristics.

Adult↗
PubMed1995

Posttransplantation lymphoproliferative disorders in solid organ recipients are predominantly aggressive tumors of host origin.

Patients immunosuppressed after organ transplantation have an increased frequency of lymphoproliferative disorders, known as posttransplantation lymphoproliferative disorders (PTLDs). In recipients of bone marrow allografts. PTLDs are often of donor origin. In only a few cases of lymphoma arising in solid-organ transplant recipients has the origin from host or donor lymphocytes been established. The authors have analyzed 11 cases of PTLD from Massachusetts General Hospital, arising in seven male and four female patients, aged 8 to 63, five with renal, four with cardiac, and two with hepatic allografts. Using the polymerase chain reaction (PCR) to investigate genetic polymorphism at the D4S174 locus on chromosome 4, the Rb1.20 locus on chromosome 13, and the D19S178 locus on chromosome 19, only one tumor (previously reported) was of donor origin, whereas 10 were of host origin. Follow-up revealed that six patients died of PTLD, one was alive with recurrent PTLD, and four were alive and well or had died of other causes, including the patient with donor-origin PTLD. Based on these cases and on a review of previously reported cases, the authors conclude that the majority of PTLDs in solid organ recipients are of host origin. There appears to be a trend toward a greater likelihood of persistent or recurrent PTLD among solid organ recipients with host-origin tumors than among those with donor-origin tumor.

Adolescent↗
PubMed1995

Extramedullary tumors of lymphoid or myeloid blasts. The role of immunohistology in diagnosis and classification.

The diagnosis of primitive hematologic malignancies in extramedullary sites (lymphoblastic lymphoma of T- or B-cell type and myeloid sarcoma) on paraffin-embedded tissue sections is difficult and often impossible because of the primitive morphology of the neoplastic cells. The authors studied 21 extramedullary tumors of lymphoid or myeloid blasts. They used a panel of 22 antibodies on frozen sections and 9 antibodies on paraffin sections to determine the spectrum of immunophenotypes and to develop a practical panel for diagnosis. All but two of the cases could be classified as lymphoid or myeloid using immunohistologic analysis. Thirteen cases were classified as lymphoblastic lymphoma/acute lymphoblastic leukemia (LBL/ALL); 10 were classified as precursor T (CD7+, CD3+/-, CD45+) and 3 as precursor B-cell (CD19+/-CD10+CD45-) type. Five cases were classified as myeloid sarcoma (CD13+ myeloperoxidase+, lysozyme+). Two LBL/ALL coexpressed either CD33 (1 case) or CD15 (1 case), and one myeloid sarcoma coexpressed TdT and CD7. One case appeared to be truly mixed lineage, coexpressing CD3 with myeloperoxidase and lysozyme, and two cases expressed no lineage-specific antigens. There were clinical differences between the three major tumor types, and within the category of T-precursor LBL/ALL, classification according to stage of thymocyte differentiation was associated with distinctive clinical features. In conclusion, the spectrum of immunophenotypes detected on frozen section was similar to that reported by flow cytometry on peripheral blood and bone marrow specimens. The most useful antigens on frozen sections were CD7 and CD3 (T cell), CD10 and CD19 (B cell), and CD13 (myeloid). TdT was coexpressed by one myeloid sarcoma and was undetectable in 40% of LBL/ALL. On paraffin sections, myeloperoxidase and lysozyme were reliable markers of myeloid lineage, but none of the markers used on paraffin sections distinguished between LBL/ALL of T- and B-precursor types. Both B-LBL/ALL and myeloid sarcomas were often CD45- on paraffin sections, which may be a obstacle in determining the diagnosis. These distinctions appear to have clinical relevance.

Adolescent↗
PubMed1995

Lymphoma of the kidney. A report of 11 cases.

We describe 11 patients first seen with symptoms or signs related to lymphoma predominantly or exclusively involving one or both kidneys. The patients were seven men and four women, aged 40-77 years (median, 67). Seven of them had one or more other prior (four), subsequent (two) or both simultaneous and subsequent (one) primary malignant or premalignant lesions. The presenting symptoms of the patients with lymphoma included local pain (five cases), loss of appetite or nausea (four cases), hematuria (two cases), weight loss (two cases) or malaise (two cases). One patient had renal failure at presentation. One lymphoma was an incidental finding at the time of aneurysm resection. Nine patients had unilateral disease; two patients had bilateral disease. Six unilateral cases were initially considered on clinical (five) or clinical and pathological (one) evaluation to be primary carcinomas of the kidney. Gross examination of nephrectomy specimens revealed fleshy or firm, yellow, tan, or gray tumors from 5.7 to 22 cm (median, 7.5) in greatest dimensions that frequently invaded perinephric fat and adjacent structures. The lymphomas were subclassified as diffuse large cell (seven cases), follicular and diffuse large cell (one case), small lymphocytic plasmacytoid (two cases), and small noncleaved cell lymphoma (non-Burkitt's type) (one case). Immunophenotyping in nine cases revealed that all were B-lineage tumors. Three patients had Ann Arbor stage I disease, three had stage II, and five had stage IV. On follow-up, ranging from 1 week to 169 months (median, 15 months), 5 patients were alive and free of lymphoma. Four patients died of progressive disease 1 week to 23 months after diagnosis. One patient is alive at 4 months but has not completed chemotherapy. One asymptomatic patient has not been treated. Renal lymphomas are predominantly large-cell lymphomas of B-lineage affecting middle-aged and older adults and often can be treated successfully. Both clinically and pathologically, they can be mistaken for carcinomas of the kidney. A high proportion of patients in this series had malignant tumors of other types.

Adult↗
PubMed1994

Uterine papillary serous carcinoma: patterns of metastatic spread.

Uterine papillary serous carcinoma (UPSC) is a distinct histologic type of endometrial cancer which is associated with a high relapse rate and poor prognosis. Between 1983 and 1993, 50 patients with UPSC of the endometrium were surgically staged. Thirty-three patients had pure UPSC and 17 had UPSC admixed with other histologies. Extrauterine disease was found in 36 women (72%). Lymph node metastases were present in 36% of women without myometrial invasion, 50% with inner one-half invasion, and 40% with outer one-half invasion. Similarly, the presence of intraperitoneal disease or positive washings did not correlate with increasing myometrial invasion. Grade and histology (mixed vs pure) were also not predictive of extrauterine disease. Patients with lymphatic/vascular space invasion (LVSI) were more likely to have extrauterine disease (85%); however, even without LVSI the incidence of extrauterine disease was 58% (P = 0.05). Unlike endometrioid adenocarcinomas, grade and depth of myometrial invasion were not significant predictors for extrauterine disease. This study reinforces the need for complete surgical staging in all patients with UPSC regardless of depth of invasion.

Aged↗
PubMed1994

Clonal analysis of posttransplant lymphoproliferative disorders, using both episomal Epstein-Barr virus and immunoglobulin genes as markers.

The authors analyzed the clonality of 15 samples of B-cell lymphoproliferations arising in eight organ allograft recipients, using immunoglobulin (Ig) gene rearrangement and the fused terminal fragment of episomal Epstein-Barr virus (EBV) DNA as independent clonal markers. The tumors arose from 1 month to 4 years following transplantation. All tumors were monomorphous, high-grade lymphomas of immunoblastic (6 cases), large-cell noncleaved (centroblastic, 1 case), or small noncleaved (1 case) type. All tumors were highly aggressive and failed to respond to decreased immunosuppression alone. Each tumor had clonal Ig gene rearrangements, including those that were negative for surface Ig. In 13 of 15 specimens (seven of the eight cases), the tumors also contained latent, circularized EBV genome. In 10 specimens from six patients, the tumors contained a single predominant form of episomal EBV DNA, indicating clonal cellular proliferation of an EBV-infected progenitor cell. Three specimens from one patient showed more than one band of episomal EBV DNA, suggesting oligoclonal expansion, despite the detection of only a single clone by Ig gene rearrangement. Linear replicating EBV DNA was not detected in any of the cases. Synchronous or metachronous specimens from multiple sites were studied in five patients, four of which were EBV-positive cases. Two patients had identical Ig gene arrangements in each specimen, indicating a single neoplastic clone at all sites; one case was EBV-negative, and the other had identical EBV episomes in each specimen. In the other three cases, apparently different Ig gene rearrangements were found at different sites. In two of these, however, the same predominant EBV episome was present at each site, indicating a common clonal origin. The third case had oligoclonal EBV bands in each specimen, with distinct patterns in at least two different sites, suggesting true multiclonality. Analysis of EBV genomes is a useful adjunct to Ig gene analysis in assessing the clonality of these lesions.

Adult↗
PubMed1994

CD20+ T-cell lymphoma. Neoplastic transformation of a normal T-cell subset.

CD20 is a 35-kDa protein that is expressed early in B-cell ontogeny and is lost during terminal B-cell differentiation into plasma cells. It is thought to be B-cell-specific. However, the CD20 antigen, detected by the monoclonal antibody L26, has been reported in some cases of T-cell lymphoma. This report describes a case of a malignant lymphoma coexpressing T-cell-lineage antigens and CD20 and characterization of a CD20+ T-cell population in the peripheral blood of healthy donors. The tumor cells were pleomorphic medium-sized cells that expressed a range of T-cell-specific antigens, including CD2, CD3, CD4, CD5, CD6, CD7, and beta F1. In addition, the tumor cells expressed CD20 on frozen (B1) and paraffin sections (L-26). Stains for other pan-B cell antigens, including CD19 and CD22, and immunoglobulin light and heavy chains were negative. To determine whether this unusual coexpression of T-cell-lineage antigens and CD20 represented aberrant antigen expression or neoplastic transformation of an unusual normal T-cell subset, the authors examined specimens of peripheral blood lymphocytes from healthy donors for evidence of a CD20+ T-cell population by using three-color immunofluorescence analysis by flow cytometry. Two distinct populations of CD20+ cells were observed in peripheral blood. One expressed bright CD20 (6.6% to 23.7%, mean 14.47% of peripheral blood lymphocytes) and other B-cell associated antigens, whereas the other expressed dim CD20 (.94% to 11.90%, mean 3.50% of peripheral blood lymphocytes) and coexpressed CD3. Approximately two thirds (52.8% to 82.3%, mean 64.1%) of the dim CD20 cells were CD8+ and one third (19.2% to 74.1%, mean 37.5) CD4+. These cells also expressed CD5 and the alpha-beta chain of the T-cell receptor and lacked CD19 and CD22. These results indicate that CD20 is expressed on some normal peripheral blood T cells. CD20 expression by T-cell lymphomas may represent neoplastic transformation of a normal subset of CD20+ T cells rather than aberrant antigen expression by neoplastic cells. The nature of the CD20 antigen on T cells and the function of the normal population remain to be determined.

Aged↗
PubMed1994

Malignant lymphoma of the testis, epididymis, and spermatic cord. A clinicopathologic study of 69 cases with immunophenotypic analysis.

We studied 69 cases of malignant lymphoma of the testis, epididymis, and spermatic cord, including 64 cases in which the tumor involved these sites at presentation and five cases in which lymphoma relapsed in the testis. The patients without prior lymphoma were 16 to 91 (mean, 56) years old. Fifty-two patients had diffuse large-cell lymphomas [seven large cleaved cell (two with follicular areas), 27 large noncleaved, two multilobated, six not otherwise specified (NOS), 10 immunoblastic]; six, small noncleaved cell; two, diffuse mixed small and large cell; one, diffuse small cleaved; one, follicular mixed small cleaved and large cell; and two, high grade, unclassified in the Working Formulation. Twenty-nine cases (55%) were stage I; five (9%), stage II; one (2%), stage III, and 18 (34%), stage IV. Forty patients (73%) achieved a complete remission; 23 had a relapse of tumor at 4 to 274 months (median, 13) and five were salvaged. At last follow-up, 20 (36%) patients were free of disease, six (11%) were alive with disease, and 29 (53%) had died of lymphoma. Features associated with longer disease-free actuarial survival (DFS) included stage I disease (p = 0.0001) and sclerosis (p = 0.0001). Among patients with stage I lymphoma, those with right-sided tumors (p = 0.005) or tumors with sclerosis (p = 0.0017) had longer DFS. Lymphomas with extensive sclerosis were all stage I (p = 0.0057). Four of five patients with secondary testicular lymphoma had extranodal primary sites. They ranged from 13 to 66 years (median, 35). Testicular relapses occurred 13-37 months after initial diagnosis. Three had diffuse large, noncleaved cell type; one, lymphoblastic and one, diffuse mixed small and large cell. Immunophenotyping showed B lineage in 33 cases and T lineage in one case. Most testicular lymphomas are B-lineage large-cell lymphomas, which frequently involve other extranodal sites at presentation and at relapse, and which often have an aggressive clinical course.

Adolescent↗
PubMed1994

Oxyphilic Sertoli cell tumor of the ovary: a report of three cases, two in patients with the Peutz-Jeghers syndrome.

Three women, aged 19, 21, and 30 years, two with the Peutz-Jeghers syndrome (PJS), had unilateral ovarian tumors composed of Sertoli cells with abundant eosinophilic cytoplasm. Electron microscopical and immunohistochemical examinations in one case supported the diagnosis of a sex cord tumor. Two patients are well 3 and 20 months postoperatively; the third was well for 15 years when recurrent tumor involving multiple intraabdominal sites was discovered. The occurrence of two of these tumors in patients with PJS and the known increased frequency of sex cord tumors in patients with this syndrome indicate an association. Sertoli cell tumor should be included in the differential diagnosis of oxyphilic ovarian tumors, particularly if there is a tubular pattern.

Adult↗
PubMed1993

Hodgkin disease, nodular sclerosis type. Implications of histologic subclassification.

BACKGROUND: The prognostic significance of the cellular composition of the nodules of Hodgkin disease, nodular sclerosis type (HDNS), is controversial. METHODS: Tumors from 79 patients with HDNS, who had a median follow-up time of 9.3 years, were studied. RESULTS: Based on British National Lymphoma Investigation criteria, 58 cases were classified as NSI (low-grade) and 21 as NSII (high-grade). The study included 24 male and 55 female patients, aged 10-57 years (mean, 27 years), who presented with Stage I (13 patients [12A, 1B]), Stage II (45 patients [40A, 5B]), or Stage III (21 patients [16A, 5B]) disease. Fifty-three patients had no relapse, 4 died of other causes, and 49 are in complete clinical remission. Twenty-six patients had progression of disease during therapy or relapsed and 17 were successfully salvaged. Overall length of survival was significantly shorter with NSII (P = 0.0001), extensive necrosis (P = 0.0034), high stage (P = 0.0058), and B symptoms (P = 0.030). Multivariate analysis showed that grade had the strongest effect on overall survival (P = 0.0042; hazard ratio = 10.19). The 5-year survival was 100% for NSI patients and 75% for NSII patients. Only B symptoms were significantly associated with risk of relapse after initial therapy (P = 0.030). For patients who relapsed, only histologic grade predicted subsequent disease-free survival (P = 0.0023; hazard ratio = 26.5). Five-year disease-free survival after first relapse was 94% for NSI patients and 11% for NSII patients. CONCLUSIONS: Patients with NSI disease who relapse have a more successful salvage and longer period of survival than do those with NSII disease. Histologic subclassification of HDNS appears clinically relevant, and consideration of histologic subtype may be important when planning therapy.

Adolescent↗
PubMed1993

Thymoma--morphologic subclassification correlates with invasiveness and immunohistologic features: a study of 122 cases.

We applied the classification system of Marino and Müller-Hermelink (Virchows Arch A Pathol Anat Histopathol 407:119-149, 1985) and Kirchner and Müller-Hermelink (Prog Surg Pathol 10: 167-189, 1989) to 122 thymic epithelial tumors to evaluate the utility of this classification and to determine its correlation with invasiveness, follicular lymphoid hyperplasia, myasthenia gravis, and immunophenotype. The majority of cases could be classified according to this scheme: eight cases (7%) were medullary thymoma, 34 cases (28%) were mixed thymoma, 20 cases (16%) were predominantly cortical (organoid) thymoma, 22 cases (18%) were cortical thymoma, 32 cases (26%) were well-differentiated carcinoma (WDC), two cases (2%) were high-grade carcinoma, and four cases (3%) were unclassifiable. We report a detailed histologic analysis of these subtypes, with criteria for diagnosis. We propose that the term "organoid thymoma" be replaced by "predominantly cortical thymoma." We also observed a previously unrecognized subtype of WDC with spindle-shaped epithelial cells, which must be distinguished from medullary thymoma. There was a strong correlation between histologic subtype and invasion (P < .0001). All medullary and mixed thymomas were either completely encapsulated (64%) or had only invasion through the capsule (36%). Organoid and cortical thymomas showed an intermediate degree of invasiveness, with 36% invasive into mediastinal fat and 14% into adjacent structures. All the cases of WDC were invasive either through the capsule (19%) or into adjacent structures (81%); five cases (16%) also showed distant spread in the form of pleural seeding (four cases) or lymph node metastasis (one case). Microscopic evaluation disclosed invasion in a substantial number of cases (28.5%) thought by the surgeon to be encapsulated. The presence of follicular lymphoid hyperplasia, either within the tumor, in the residual normal thymus, or in both, was a significant predictor of myasthenia gravis (P < .0015); there was an increased association of myasthenia gravis and thymomas with cortical type epithelium (organoid thymoma, cortical thymoma, and WDC). There was no correlation between epithelial cell morphology and antigen expression; however, the immunophenotype of the associated lymphocytes corresponded closely to the morphologic classification of the thymomas. The Müller-Hermelink morphologic classification can be applied to most cases of thymoma and strongly predicts invasive behavior. The correlation of lymphocyte immunophenotype with the morphologic classification of the epithelial cells suggests that it is biologically accurate.

Adolescent↗