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Lymphomas do not only only arise in lymph nodes. Many arise in non-lymphoid sites such as the skin and gastrointestinal tract. The gastrointestinal tract is the most common extra-nodal site for follicular lymphomas, the most common nodal based type of lymphoma.

Cutaneous Lymphomas
Enteropathy-type T-cell Lymphoma
Urinary Bladder Lymphomas
MALTOMA (Mucosal Associated Lymphoid Tissue Lymphomas)
Splenic Marginal Zone Lymphoma


Primary adrenal lymphoma.

Harris GJ, Tio FO, Von Hoff DD.

Department of Medicine, University of Texas Health Science Center, San Antonio 78284.

Cancer 1989 Feb 15;63(4):799-803 Abstract quote

A case of non-Hodgkin's lymphoma (large cell immunoblastic sarcoma) arising as a primary malignancy in the adrenal gland is reported and the literature concerning this unusual tumor is reviewed. Although extremely rare, it is possible to make the diagnosis preoperatively.

Therefore, primary adrenal lymphoma should be included in the differential diagnosis of a suprarenal mass.

Non-Hodgkin's lymphoma confined to the adrenal glands presenting with Addison's disease.

Khan S, Raby N, Michell M.

Department of Radiology, King's College Hospital, London.

Clin Radiol 1990 Jul;42(1):63-4 Abstract quote

We report an unusual case of Addison's disease which is unique in being diagnosed ante-mortem as being due to Non-Hodgkin's lymphoma of the adrenal glands. Both ante- and post-mortem examinations showed the lymphoma to be entirely confined to the adrenals.

Non-Hodgkin's lymphoma of the adrenal gland.

Choi CH, Durishin M, Garbadawala ST, Richard J.

Department of Pathology, Doctors Hospital, Massillon, OH 44646.

Arch Pathol Lab Med 1990 Aug;114(8):883-5 Abstract quote

Primary non-Hodgkin's lymphoma of the adrenal gland was diagnosed in a 64-year-old woman. The histologic and immunohistologic features are characteristic of a large-cell noncleaved lymphoma (B-cell origin). The patient died 3 weeks after right-sided adrenalectomy. The patient's family refused an autopsy.

Malignant lymphoma of the adrenal gland: its possible correlation with the Epstein-Barr virus.

Ohsawa M, Tomita Y, Hashimoto M, Yasunaga Y, Kanno H, Aozasa K.

Department of Pathology, Osaka University Medical School, Japan.

Mod Pathol 1996 May;9(5):534-43 Abstract quote

Initial manifestation of malignant lymphoma in the adrenal gland is a rare event, and clinical and pathologic features are not fully understood.

We conducted a nationwide study in Japan, and 20 patients with malignant lymphoma that showed initial and main manifestation in the adrenal gland were identified. Clinical and pathologic findings were summarized. In addition, the presence of the Epstein-Barr virus (EBV) genome in the tumor cells was examined by using polymerase chain reaction (PCR) and in situ hybridization (ISH), together with the immunohistochemical evaluation of the expression of latent membrane protein-1 (LMP-1). There were 13 men and seven women; their ages at admission ranged from 40 to 87 years (median, 65 yr). Fever, anemia, and elevation of lactic dehydrogenase levels were the common presenting findings. One patient had acquired immunodeficiency syndrome. Adrenal tumors were bilateral in 15 patients and unilateral (all in the left site) in five. Prognosis was very poor; all but two patients died within 1 year after admission. Histologically diffuse large cell type was the commonest type (14 specimens). Immunohistologically, 16 specimens were B-cell type, and one was T-cell type. Another three specimens showing no positive reaction for any antibodies were also judged as B-cell type on purely morphologic grounds. Prominent intravascular proliferation of tumor cells was found in five patients. PCR for EBV genomes gave positive results in five patients; the virus was subtyped as A in three patients and as B in two.

The ISH provided positive signals in nine samples, including all five specimens positive for PCR. Four of the nine cases with detectable EBV by PCR and/or ISH expressed LMP-1. The present study shows that adrenal lymphoma is EBV associated and has a B-cell phenotype.

Primary adrenal lymphoma: gallium scintigraphy and correlative imaging.

Truong B, Jolles PR, Mullaney JM.

Department of Radiology, Medical College of Virginia Hospitals; Virginia Commonwealth University, Richmond 23298, USA.

J Nucl Med 1997 Nov;38(11):1770-1 Abstract quote

Primary adrenal lymphoma is a rare entity, with only 16 cases reported in the last 40 yr. Although 67Ga scintigraphy has been extensively used to evaluate patients with other types of lymphomas, there are no reports of its use in patients with this disease entity.

A man with primary adrenal lymphoma and no evidence of extraadrenal spread who was evaluated from presentation to remission with gallium scintigraphy and CT is presented. Gallium scintigraphy was valuable in assessing response to therapy.

Clinically silent primary adrenal lymphoma: a case report and review of the literature.

Wang J, Sun NC, Renslo R, Chuang CC, Tabbarah HJ, Barajas L, French SW.

Department of Pathology, Harbor-UCLA Medical Center, Torrance, California 90509, USA.

Am J Hematol 1998 Jun;58(2):130-6 Abstract quote

Primary adrenal lymphoma (PAL) is extremely uncommon.

We describe a case of clinically silent non-Hodgkin's B-cell lymphoma of diffuse large cell type with exclusive left adrenal localization. The tumor was discovered by computed tomography (CT) as a 2.5-cm dense mass and diagnosed at autopsy. Literature concerning this unusual neoplasm is reviewed. During the early stage, particularly when the lesion is small, PAL is likely to be missed.

This unusual entity should be included in the differential diagnosis of adrenal masses so that early diagnosis may be made and intervention might dramatically affect the clinical outcome.

A case of primary bilateral adrenal lymphoma with partial adrenal insufficiency.

Lee KS, Chung YS, Park KH, Kim HS, Kim HM.

Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea.

Yonsei Med J 1999 Jun;40(3):297-300 Abstract quote

Unilateral or bilateral non-Hodgkin's lymphomas arising primarily in the adrenal glands are extremely rare. These lymphomas are usually present with large, bilateral adrenal masses with or without lymphadenopathy, and may be accompanied by adrenal insufficiency in some cases. A review of the literature indicates that patients with primary lymphoma of the adrenal glands usually do not have disease elsewhere, and if present, it is frequently extranodal.

We report here an unusual case of primary bilateral adrenal lymphoma with partial adrenal insufficiency.


An Immunophenotypic and Molecular Study of Primary Large B-Cell Lymphoma of Bone

David Huebner-Chan, M.D., Bernie Fernandes, M.B., Ch.B., Guisheng Yang, M.D. and Megan S. Lim, M.D., Ph.D.

Department of Pathology (DH-C)Kaiser Permanente Medical Center, Anaheim, California; Department of Pathology (BF), Mount Sinai Hospital; and Sunnybrook and Women’s College Health Sciences Center (GY, MSL), University of Toronto, Toronto, Canada


Mod Pathol 2001;14:1000-1007 Abstract quote

Primary non-Hodgkin’s lymphomas of bone (PNHLB) is a rare form of extranodal lymphoma.

Many studies have reported the clinical, radiologic, and histopathologic characteristics of PNHLB; however, their molecular features have not been well studied. In this report, we present the immunophenotypic and molecular characteristics of 20 primary large B-cell lymphoma (PLBCL) of bone from 20 adults. Most demonstrated centroblastic morphology, with the majority exhibiting nuclear multilobation. One case (5%) demonstrated anaplastic features with strong CD30 expression but was ALK-1 negative. BCL-6 expression was seen in 6 of 20 cases, and strong p53 protein expression was seen in 11 of 20 (55%) cases. The majority of cases analyzed (13/18 = 72%) demonstrated a clonal B-cell process by IgH gene rearrangement studies. Of the five cases that did not demonstrate a clonal population, two expressed BCL-6 protein. No cases demonstrated a bcl-2/JH rearrangement, but BCL-2 protein expression was seen in 11 of 20 (55%) cases.

In summary, primary lymphoma of bone is largely a non-Hodgkin’s lymphoma of large B-cell type. Our studies demonstrate that p53 and BCL-2 expression may play a role in the pathogenesis of PLCBL of bone. In addition, a subset of the cases are of putative germinal center B-cell origin based on the expression of BCL-6 protein and may be genetically distinct from follicle center lymphomas. The results provide evidence for molecular heterogeneity within primary large B-cell lymphomas of bone.

GASTROINTESTINAL TRACT Most common location of extranodal follicular lymphomas

Lymphoid lesions of the gastrointestinal tract: a histologic, immunophenotypic, and genotypic analysis of 49 cases.

O'Sullivan MJ, Ritter JH, Humphrey PA, Wick MR.

Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St. Louis, Missouri 63110-1093, USA.

Am J Clin Pathol 1998 Oct;110(4):471-7 Abstract quote

The diagnosis of gastrointestinal (GI) lymphoid infiltrates can be challenging when based only on conventional microscopic assessment. When marked cytologic atypia is present, a diagnosis of malignant neoplasm is readily made; however, the distinction between a low-grade malignant neoplasm and a reactive process is much more difficult. If unfixed tissue is available, immunohistologic or genotypic methods that are usually aimed at defining B-lymphocytic monotypism can be applied. However, paraffin-embedded tissue has generally been deemed unsuitable for these techniques.

We assessed the value of a panel of immunohistochemical stains and a seminested polymerase chain reaction (PCR) for the analysis of lymphoid infiltrates in routinely processed GI biopsy specimens from 49 archival cases, including morphologically benign, indeterminate, and overtly malignant lesions.

Clinical outcome was used as the retrospective diagnostic standard; end points were death (of lymphomatous disease or otherwise) and clinical evidence of lymphoma. According to light microscopic criteria, 19 cases were classified as benign, 17 as malignant, and 13 as atypical. Immunophenotyping correctly identified 28 of 31 benign and 14 of 18 malignant lesions (7 cases had an indeterminate immunoprofile). Genotypic analysis correctly identified 12 of 18 malignant and 29 of 31 benign lesions, but spurious monoclonal bands were produced by PCR amplification of 2 of the latter 31 cases.

No single technique exists for correct categorization of all paraffin-embedded specimens of GI lymphoid infiltrates. We recommend a sequential approach to the use of available diagnostic modalities.

Increased Incidence of Follicular Lymphoma in the Duodenum

Tadashi Yoshino, M.D., Ph.D.; Kenji Miyake, M.D., Ph.D.; Koichi Ichimura, M.D.; Tomohiko Mannami, M.D.; Nobuya Ohara, M.D., Ph.D.; Shuji Hamazaki, M.D., Ph.D.; Tadaatsu Akagi, M.D., Ph.D.

From the Departments of Pathology (T.Y., K.M., K.I., T.M., N.O., T.A.) and Central Laboratory (S.H.), Okayama University Medical School, Japan.

Am J Surg Pathol 2000;24:688-693 Abstract quote

The incidence of indolent lymphomas in the lymph nodes and extranodal regions is quite different. Follicular lymphoma (FL) is most common in the nodes, and it seems to be least common in the gastrointestinal (GI) tract, where mucosa-associated lymphoid tissue lymphoma arises most frequently.

The authors report that the incidence of FL is unexpectedly high in the duodenum compared with other portions of the GI tract. FL was detected in only eight of 222 cases of GI lymphoma (3.6%). However, five cases of FL arose in the duodenum, which accounted for 38.5% of 13 duodenal lymphomas. Only in two patients did FL arise in either the stomach or the colorectum, and in the remaining patients FL was widespread with lymphomatous polyposis.

Duodenal FL was composed of neoplastic follicles with small cleaved cells in dominance, and the immunophenotype of the lymphoma cells was CD10+, BCL-2+, CD20+, CD75+, CD79+, CD3–, CD5–, cyclin D1–, CD23–, and CD45RO–.

All the patients were women age 37 to 66 years (average age, 52.4 yrs). In all patients the lymphoma was present around the ampulla of Vater, and four of five patients showed multiple small-size polyps. Although lymphoma cell infiltration was confined to the submucosa in the four patients examined, the regional lymph nodes were involved partially in two patients without distant metastasis. All patients are alive at 2 to 50 months of follow up (average, 27 mos), which is comparable with the prognosis for indolent nodal lymphomas.

These results suggest that the duodenum has a distinct background of histogenesis of the lymphomas and that biopsy specimens from the duodenum with multiple polyps should be examined carefully.

Primary Follicular Lymphoma of the Gastrointestinal Tract
A Clinical and Pathologic Study of 26 Cases

Jinru Shia, M.D. ; Julie Teruya-Feldstein, M.D. ; Dorothy Pan, M.D. ; Abhijith Hegde, M.D. ; David S. Klimstra, M.D. ; R. S. K. Chaganti, Ph.D. ; Jing Qin, Ph.D. ; Carol S. Portlock, M.D. ; Daniel A. Filippa, M.D.

From the Departments of Pathology (J.S., J.T.-F., D.S.K., D.A.F.), Medicine (D.P., A.H., R.S.K.C., C.S.P.), and Biostatistics (J.Q.), Memorial Sloan-Kettering Cancer Center, New York, NY, U.S.A.

Am J Surg Pathol 2002;26:216-224 Abstract quote

Although the gastrointestinal tract represents the most common site of extranodal lymphoma, primary follicular lymphoma of the gastrointestinal tract is an uncommon and poorly defined disease.

We report the clinical and pathologic features of 26 patients with primary gastrointestinal follicular lymphoma. Ten of 26 patients (38.5%) were stage IIE, and 16 patients (61.5%) were stage IE. Of the 26 patients, 13 were female and 13 were male. The age range was 26–81 years (median 54.5 years). Abdominal pain was the most common presenting symptom, seen in 12 of 24 patients (50%). Nodularity of the mucosal surface was the most common endoscopic finding, seen in 10 of 14 patients (71.4%). The majority of cases (22 of 26, 84.6%) involved small bowel, four involved colorectum alone, and two involved the ileocecal valve. Within the small bowel the duodenum was the most commonly involved site (10 cases). Transmural involvement by follicular lymphoma was identified in 11 of the 16 patients who underwent surgical resection; five showed involvement of mucosa and submucosa only. The most common histologic grade was grade 1. Thirteen of 26 cases were grade 1, ten grade 2, and three grade 3. Twenty-one of 26 cases showed a predominantly follicular growth pattern, four mixed follicular and diffuse, and one predominantly diffuse. All cases were positive for CD20 and BCL2 and negative for CD3, CD5, CD23, CD43, and cyclin D1. Twenty-four of 26 were positive for CD10. Four of four cases showed cytogenetic or molecular genetic evidence of t(14;18). Initial treatment modalities included surgery plus chemotherapy (nine cases), surgery alone (seven cases), chemotherapy alone (four cases), observation alone (four cases), and chemotherapy and abdominal radiation (one case). One case presented with rectal polyps and was treated with polypectomy. A complete response was observed in 15 of 22 cases that received treatment, and of the 15 cases, five recurred 27–60 months after the initial diagnosis. Recurrence and progression were associated with histologic transformation to diffuse large cell lymphoma in one case. No significant correlation was identified between treatment response and various clinical and pathologic features.

Overall, none of the 26 patients died of lymphoma. One patient died of a concomitant pancreatic carcinoma. Of the remaining 25 patients, 14 were disease free and 11 were alive with disease at a mean follow-up of 43 months. The estimated 5-year disease-free survival was 62%, and median disease-free survival was 69 months. The estimated 5-year relapse-free survival was 54%, and the median relapse-free survival was 63 months.

CD10 Expression in Primary Intestinal Large B-Cell Lymphomas.

Go JH, Yang WI, Ree HJ.

Department of Pathology, Dankook University College of Medicine, Cheonan-si, Chungchungnam-do, Korea (Dr Go); the Department of Pathology, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea (Dr Yang); and the Department of Diagnostic Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea (Dr Ree).

Arch Pathol Lab Med 2002 Aug;126(8):956-960 Abstract quote

Objective.-To determine the prevalence and clinical significance of a follicular origin in primary intestinal large B-cell lymphomas.

Design.-We performed immunohistochemistry for detection of CD10 and Bcl-6 in paraffin-embedded tissue sections from 32 patients who had surgically resected primary intestinal lymphomas. We then correlated the expression of CD10 protein with various clinical parameters.

Results.-Fifteen of 32 cases (47%) were CD10(+), and CD10 positivity was correlated with intense Bcl-6 positivity. There were no significant differences in age, sex, serum lactate dehydrogenase level, and performance status between the CD10(+) and CD10(-) groups. Patients with CD10(+) lymphoma more frequently presented with stage I(2) disease compared with those with CD10(-) lymphoma (P =.03). The response to treatment was significantly better in the CD10(+) group than in the CD10(-) group (P =.02). An analysis of survival rates showed a trend for a longer overall survival duration in the CD10(+) group compared with the CD10(-) group, although this was not statistically significant (P =.05).

Conclusions.-We propose that more than half of primary intestinal large B-cell lymphomas have a germinal center cell origin. We also suggest that CD10 expression may be a prognostic indicator in patients with primary intestinal large B-cell lymphomas.


Lymphomas of the oral cavity: Histology, immunologic type, and incidence of Epstein-Barr virus infection.

Solomides CC, Miller AS, Christman RA, Talwar J, Simpkins H.

Department of Pathology and Laboratory Medicine, Temple University School of Medicine, Philadelphia, PA.

Hum Pathol 2002 Feb;33(2):153-7 Abstract quote

The purpose of this study was to determine the histologic class and immunologic phenotype of lymphomas presenting initially in the oral cavity and whether this correlated to a high incidence of Epstein-Barr virus (EBV) infection as has been reported with lymphomas in the nasal cavity.

Seventy-one cases of oral lymphomas from the oral pathology referral service were analyzed retrospectively. They were classified according to the Revised European American Lymphoma (REAL) classification system using routine immunohistochemistry. EBV infection was determined by detection of early viral RNA sequences (EBER) and latent membrane protein (LMP-1) expression.

Only non-Hodgkin's lymphomas were observed, with a female predominance of 2:1. They were primarily of B-cell origin and histologically classified mainly as large B-cell type (68%); T-cell lymphomas were rare (8%). EBV infection was observed in 14% of the B-cell lymphomas, an incidence rate higher than that reported in studies of B-cell lymphomas not located in the oral cavity but not as high as that observed in pleomorphic T-cell lymphomas (all sites, 36%) or nasal cavity T-cell lymphomas (nearly 100%). Interestingly, EBV proliferation did not correlate with expression of either Bcl-2 or p53.


Primary Lymphoma of Peripheral Nerve Report of Four Cases

Joseph Misdraji, M.D.; Yasushi Ino, M.D.; David N. Louis, M.D.; Andrew E. Rosenberg, M.D.; E. Antonio Chiocca, M.D., Ph.D.; Nancy L. Harris, M.D. Spencer PS, Schaumburg HH, eds.

From the James Homer Wright Pathology Laboratories of Massachusetts General Hospital and Department of Pathology (J.M., Y.I., D.N.L., A.E.R., N.L.H.), Harvard Medical School; and the Neurosurgery Service of Massachusetts General Hospital (Y.I., D.N.L., E.A.C.), Boston, Massachusetts, U.S.A.

Am J Surg Pathol 2000;24:1257-1265 Abstract quote

Lymphoma presenting as a solitary tumor of peripheral nerve is exceedingly rare, with only six previously reported cases. The authors describe an additional four cases of primary lymphoma of peripheral nerve involving the sciatic nerve (two cases), the radial nerve, and the sympathetic chain and spinal nerve.

The patients were two men and two women with an average age of 55.5 years. All tumors were high-grade B-cell lymphomas. Two patients experienced relapse of disease with involvement of other nervous system sites and died of lymphoma. One patient is alive with stable local disease at 57 months. The fourth patient is alive with no evidence of disease at 54 months. Expression of neural cell adhesion molecule (CD56) has been reported to correlate with an increased incidence of central nervous system involvement in peripheral T-cell lymphoma; all their cases were CD56 negative. Recent reports indicate a high proportion of primary brain lymphomas show loss of CDKN2A/p16 gene expression. Therefore, CDKN2A/p16 was evaluated in their patients both by polymerase chain reaction and by immunohistochemistry for the p16 protein. The authors found homozygous deletion of the CDKN2A/p16 gene in one of three patients studied, confirmed immunohistochemically by absent staining for p16. The fourth patient showed absent staining for p16, suggesting inactivation of the gene in this case as well. The two patients with p16 loss both died of lymphoma, whereas the two patients with normal p16 expression are alive.

Primary lymphoma of peripheral nerve is a rare neoplasm, usually of large B-cell type, has a variable prognosis, and appears to have less consistent loss of p16 expression than primary central nervous system lymphoma.


Ovarian Non-Hodgkin’s Lymphoma: A Clinicopathologic Study of Eight Primary Cases

Russell Vang, M.D., L. Jeffrey Medeiros, M.D., Roger A. Warnke, M.D., John P. Higgins, M.D. and Michael T. Deavers, M.D.

Stanford University Medical Center (RV, RAW, JPH), Department of Pathology (Laboratory of Surgical Pathology), Stanford, California; and The University of Texas-M.D. Anderson Cancer Center (JM, MTD), Houston, Texas

Mod Pathol 2001;14:1093-1099 Abstract quote

Primary (localized) non-Hodgkin’s lymphoma (NHL) of the ovary is rare.

We studied eight cases of primary ovarian NHL to better understand the clinicopathologic and immunophenotypic features of these tumors. The patients ranged in age from 29 to 62 years (mean 47 years). Pelvic complaints were the most common symptoms; however, three of eight neoplasms were discovered incidentally. All tumors were unilateral and Ann Arbor stage IE. The three incidental NHL were microscopic (largest 1.2 cm), whereas the grossly evident lesions ranged from 7.5 to 20 cm (mean 13.3).

Each tumor was classified according to the World Health Organization Classification as follows: diffuse large B-cell lymphoma (three cases), follicular lymphoma (two cases), Burkitt lymphoma (one case), T-cell anaplastic large cell lymphoma (one case), and precursor T-lymphoblastic lymphoma (one case). Six tumors were of B-cell lineage, and two tumors were of T-cell lineage. All three diffuse large B-cell lymphomas were positive for BCL-6, two were positive for CD10, and two were positive for BCL-2. Estrogen and progesterone receptors were negative in all NHLs assessed. Patients were treated by various combinations of surgery, chemotherapy, and radiotherapy. Clinical follow-up ranged from 1.3 to 11.7 years (mean 5.2) and all patients were alive without disease at last follow-up.

We conclude that most patients with primary ovarian NHL present with symptoms attributable to an ovarian mass, but in a subset of patients ovarian NHL may be detected incidentally. With appropriate therapy, patients appear to have a favorable prognosis although follow-up is short for some patients in this study.


Pyothorax-associated lymphoma: a review of 106 cases.

Nakatsuka S, Yao M, Hoshida Y, Yamamoto S, Iuchi K, Aozasa K.

Department of Pathology, Osaka University Graduate School of Medicine, Suita, Japan.

J Clin Oncol 2002 Oct 15;20(20):4255-60 Abstract quote

PURPOSE: Pyothorax-associated lymphoma (PAL) is a non-Hodgkin's lymphoma developing in the pleural cavity after a long-standing history of pyothorax. Full details of PAL are provided here.

PATIENTS AND METHODS: Clinical and pathologic findings were reviewed in 106 patients with PAL collected through a nationwide survey in Japan.

RESULTS: Age of the patients with PAL was 46 to 82 years (median, 64 years), with a male/female ratio of 12.3:1. All patients had a 20- to 64-year (median, 37-year) history of pyothorax resulting from artificial pneumothorax for treatment of pulmonary tuberculosis (80%) or tuberculous pleuritis (17%). The most common symptoms on admission were chest and/or back pain (57%) and fever (43%). Laboratory data showed that the serum neuron-specific enolase level was occasionally elevated (3.55 to 168.7 ng/mL; median, 18.65 ng/mL), suggesting a possible diagnosis of small-cell lung cancer. Histologically, PAL usually showed a diffuse proliferation of large cells of B-cell type (88%). In situ hybridization study showed that PAL in 70% of the patients was Epstein-Barr virus (EBV)-positive. PAL was responsive to chemotherapy, but the overall prognosis was poor, with a 5-year survival of 21.6%.

CONCLUSION: This study established the distinct nature of PAL as a disease entity. PAL is a non-Hodgkin's lymphoma of exclusively B-cell phenotype in the pleural cavity of patients with long-standing history of pyothorax, and is strongly associated with EBV infection. Development of PAL is closely related to antecedent chronic inflammatory condition; therefore, PAL should be defined as malignant lymphoma developing in chronic inflammation.


Excessive survivin expression in thyroid lymphomas.

Sugawara M, Matsuzuka F, Fukata S, Kuma K, Moatamed F, Haugen BR.

Division of Endocrinology and Metabolism, Molecular Pathology Division, Department of Pathology and Laboratory Medicine, Greater Los Angeles Veteran's Affairs Medical Center and UCLA School of Medicine, 90073, USA.

Hum Pathol 2002 May;33(5):524-7 Abstract quote

Thyroid lymphoma occurs most commonly in the thyroid glands with a background of Hashimoto's thyroiditis. Therefore, it is occasionally difficult to distinguish lymphoma from Hashimoto's thyroiditis because of some cellular and histologic similarities.

We have examined whether survivin or human telomerase reverse transcriptase (hTERT) expression can differentiate between the 2 disorders. Surgically removed tissue samples from 6 patients with thyroid lymphoma and 6 patients with Hashimoto's thyroiditis were analyzed for mRNA levels of survivin and hTERT by real-time quantitative reverse-transcription polymerase chain reaction. Expression of survivin protein was examined by immunohistochemical stain using a polyclonal antibody. Survivin mRNA levels were greater in thyroid lymphoma than in Hashimoto's thyroiditis: 49.1 +/-36.4 versus 6.6 +/-2.7 pg/ng rRNA (mean +/- SD) (P <0.005). Immunohistochemical stain confirmed an abundance of survivin protein in lymphoid cells of thyroid lymphoma. The amount of hTERT mRNA did not differ in the 2 disorders.

Our study shows that measuring survivin mRNA levels or immunohistochemistry of the protein expression can be useful to aid the diagnosis of thyroid lymphoma when histologic diagnosis is difficult.


Focal follicular features in tonsillar diffuse large B-cell lymphomas: follicular lymphoma with diffuse areas or follicular colonization.

Ree HJ, Kikuchi M, Lee SS, Ohshima K, Yang WI, Ko YH, Cho EY, Rhee JC.

Department of Diagnostic Pathology, Samsung Medical Center, Seoul, Korea.

Hum Pathol 2002 Jul;33(7):732-40 Abstract quote

Focal follicular features in diffuse large B-cell lymphomas (DLBCLs) are bound to raise the question of follicular lymphoma (FL) with diffuse areas, because the diagnosis of FL is based on the presence of follicular areas, even though focal.

We report 7 cases of primary tonsillar DLBCLs with focal follicular features that presented with morphologic, immunohistochemical, and biological features distinct from those of FL. Histologically, these tumors were characterized by involvement of pericryptal follicles with adjacent dominant diffuse areas. Monomorphous large tumor cells were evenly spaced with abundant, often clear cytoplasm, and blastoid nuclei often with a delicate nuclear membrane. Importantly, residual germinal centers (GCs) were present in the form of either an intrafollicular GC remnant or an isolated GC in the midst of diffuse tumor. An extrafollicular and/or parafollicular growth pattern was also observed. Bcl-6 staining revealed a predominantly sporadic occurrence of Bcl-6(+) cells, comprising <50% of tumor cells, and none displayed diffusely dense collections (>75%) of Bcl-6(+) tumor cells characteristic of the GC or FL. Staining for CD10 was negative in 6 cases. Five of 7 patients were younger than 60, the median age of other patients with primary tonsillar DLBCL.

No extratonsillar involvement was seen at 18 months after diagnosis. After chemotherapy or radiotherapy, complete remission was achieved with ease in all patients, but 2 patients who were treated with chemotherapy alone relapsed at 24 and 30 months. In conclusion, tonsillar DLBCL includes a small (10%) but distinct subgroup that warrants distinction from FL with predominant diffuse areas or de novo DLBCL.

It appears that the focal follicular features in tonsillar DLBCL likely represent follicular colonization of marginal zone B-cell lymphoma, probably high-grade, if the possibility of FL is excluded.

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