AIMS--To investigate whether immunohistochemical staining using p53 and/or bcl-2 distinguishes between florid Hashimoto's thyroiditis and low grade mucosa associated lymphoid tissue (MALT) lymphoma of the thyroid. METHODS--Ten cases of Hashimoto's thyroiditis and eight of primary thyroid lymphoma were stained with monoclonal antibodies directed against p53 and bcl-2. RESULTS--In Hashimoto's thyroiditis most small lymphoid cells in mantle zones, within the thyroid parenchyma and in lymphoepithelial lesions expressed bcl-2 protein. Very occasional centroblasts in reactive germinal centres were positive for p53, but all other lymphoid cells from cases of Hashimoto's disease were negative for p53. In diffuse, low grade lymphomas bcl-2 protein was uniformly expressed by most tumour cells. However, low grade lymphomas with a follicular pattern did not express bcl-2. The diffuse, low grade lymphomas were negative for p53, while occasional larger cells in the follicular subtype were positive. Both high grade lymphomas were bcl-2 negative but strongly p53 positive. CONCLUSIONS--This study indicates that there is an inverse correlation between p53 and bcl-2 immunostaining in thyroid lymphomas (low grade lymphomas: bcl-2 positive, p53 negative; high grade lymphomas: bcl-2 negative, p53 positive). Furthermore, immunohistochemical staining for bcl-2 and p53 proteins does not distinguish florid Hashimoto's thyroiditis from diffuse, low grade thyroid lymphoma.
We present here two cases of incidental extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) showing prominent plasma cell differentiation associated with Hashimoto’s thyroiditis (HT). Histological examination demonstrated that both lesions exhibited HT including lymphoplasmacytic infiltration with the formation of germinal centers, destruction of the normal thyroid follicular architecture, Hürthle cell changes, and squamous metaplasia. The dominant tumor nodules of both cases contained large, well-circumscribed but unencapsulated aggregation of mature plasma cells and scattered centrocyte-like cells (CCL-cells). Both lesions contained a few lymphoepithelial lesions. Moreover, immunohistochemical study demonstrated that plasma cells and CCL-cells of these two lesions contained monotypic intracytoplasmic kappa light chain. Other small B-cell lymphomas, plasmacytoma and plasmablastic lymphoma were excluded using stains for CD5, CD10, CD23, CD43, CD56. Cyclin D1, human herpes virus type-8.
Thyroid gland; MALT type lymphoma; Hashimoto’s thyroiditis; Plasma cell granuloma; Immunohistochemistry
Primary thyroid gland lymphomas are uncommon tumours that occur in the setting of lymphocytic thyroiditis or Hashimoto's disease in almost all cases. In this condition a distinction between an inflammatory lymphoid infiltrate and a low grade lymphoma may be extremely difficult and precise criteria are necessary for a correct diagnosis.
Patient and methods
We report a case of a minute focus of primary extranodal marginal zone B-cell lymphoma (EMZBCL), incidentally discovered in a 63-year-old man with Hashimoto thyroiditis (HT) and diagnosed by means of polymerase chain reaction (PCR) after laser capture microdissection.
The histological examination of surgical specimen confirmed the diagnosis of HT and showed a minute focus of dense lymphoid infiltrate (less than 4 mm in diameter), composed by centrocyte-like cells forming MALT balls. Immunoistochemistry was not useful. A microscopic focus of EMZBCL was suspected on the basis of morphological features. PCR assays revealed the rearrangement of the heavy chain of immunoglobulins only in the microdissected suspicious area, confirming the diagnosis of EMZBCL.
Our finding suggests that in cases of autoimmune thyroiditis a careful examination of the thyroid specimen is warranted, in order to disclose areas or small foci of lymphomatous transformation. Furthermore, in difficult cases with doubtful immunohistological findings, ancillary techniques, such as molecular studies, are necessary for a conclusive diagnosis.
Primary thyroid lymphoma is a rare malignancy, representing 2–8% of all thyroid malignancies and 1–2% of all extranodal lymphomas. The majority of cases concern non-Hodgkin's lymphoma of B cell origin, following by Hodgkin's disease, T cell lymphomas and rarely marginal zone B-cell mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas have been associated with long-standing autoimmune Hashimoto's thyroiditis. We present the case of a 44-years-old woman with thyroid MALT lymphoma in the background of multinodular goiter of autoimmune origin.
thyroid MALT lymphoma; thyroiditis Hashimoto.
The majority of lymphomas arising in the thyroid gland are MALT 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 clinicopathological features at this site are not well characterised, leading to difficulties in diagnosis and clinical management. We have addressed this problem by studying the clinical, morphological, 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 MALT lymphoma at this site. Furthermore, 13 of 18 cases for whom sufficient evidence was available had clinical and/or histological evidence of chronic lymphocytic thyroiditis. Analysis of genetic and immunohistochemical features identified two distinct groups. In one group, similar to typical adult follicular lymphoma, cases carried a t(14;18)/IGH-BCL2 and/or expressed Bcl2, and were mostly CD10-positive and of WHO grade 1-2. 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 two 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 morphological, immunophenotypic and genetic characteristics of follicular lymphoma presenting in the thyroid gland should aid both diagnosis and clinical management.
Thyroid gland; follicular lymphoma; extranodal lymphoma
Introduction. Primary thyroid lymphomas constitute up to 5% of all thyroid malignancies and can be divided into non-Hodgkin's lymphomas (NHLs) of B- and T-cell types, as well as Hodgkin's lymphomas. Mucosa-associated lymphoid tissue (MALT) lymphomas are a relatively recently recognized subset of B-cell NHLs, and they are listed as extranodal marginal zone lymphomas according to the revised European-American lymphoma classification. Case Report. We report an uncommon case of a 44-year-old man, who noted a painless, growing mass on right side of his neck of the three-month duration. Thyroid profile was within normal limits. FNAC showed lymphocytic thyroiditis. The patient underwent a right hemithyroidectomy. The histologic examination and the immunohistochemistry showed an extra nodal marginal B-cell type maltoma (malt lymphoma). CHOP chemotherapy with rituximab was given. The clinical course has been favourable in the first year of followup, with no evidence of local or systemic recurrence of the disease. Discussion. Marginal zone lymphoma encompasses a heterogeneous group of B-cell tumours that variously arise within the lymph nodes, spleen, or extranodal tissues. A case of maltoma of thyroid is presented for its rarity and diagnostic dilemmas. Conclusion. Maltomas are slow-growing lymphomas. The optimal treatment and followup of patients with thyroid maltomas remain controversial at present.
Primary gastric T cell lymphoma is rare and mostly of large cell type. In this paper, we present a case of gastric T cell lymphoma morphologically similar to the gastric marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT). Morphologically, the cells are small with abundant clear cytoplasm. Lymphoepithelial lesions are readily identified with diffuse destruction of gastric glands. Immunohistochemically, the neoplastic cells are CD3+/CD4+/CD8−/Granzyme B−. Molecular studies revealed monoclonal T cell receptor γ gene rearrangement. Clinically, the patient responded initially to four cycles of R-CHOP, but then progressed. Because peripheral T cell lymphoma is usually associated with a poor prognosis, whereas marginal zone B cell lymphoma is an indolent lymphoproliferative disorder, this morphologic mimicry should be recognized and completely investigated when atypical small lymphoid infiltrates with lymphoepithelial lesions are encountered in the stomach.
Primary gastric T cell lymphoma; MALT; H. pylori; HTLV-1
Background: Hashimoto’s thyroiditis (HT) is a risk factor for thyroid lymphoma, and clonal B cell populations in HT support this link. The literature on B cell clonality in HT is controversial.
Aims: To identify clonal B cell populations in HT and to assess their usefulness in differentiating HT from mucosa associated lymphoid tissue (MALT) lymphoma and predicting future development of lymphoma.
Methods: DNA from formalin fixed, paraffin wax embedded blocks of thyroid specimens from 10 patients with HT and two thyroid MALT lymphomas was analysed for B cell clonality by seminested polymerase chain reaction (PCR) using FRIII/LJH and FRIII/VLJH primers to amplify the IgH gene VDJ region. In one case, PCR products were sequenced. Immunohistochemistry was performed by labelled streptavidin–biotin technique using antibodies to: CD45, CD45RO, CD3, CD20, and cytokeratin.
Results: The histopathological and clinical findings were characteristic of HT. Clonal bands were seen in three and a polyclonal smear pattern was seen in seven cases. The clonal bands in HT were associated with a background smear, and could not be reproduced from other blocks from the same case or from deeper sections of the same block. The clonal bands in thyroid lymphomas were not associated with a background smear and were reproducible. None of the patients with clonal B cells has developed malignant lymphoma during a follow up of 10–13 years.
Conclusions: B cell clonal bands in HT have different features from those in lymphoma (non-pure and non-reproducible) and do not predict future development of lymphoma.
Hashimoto’s thyroiditis; clonality; immunoglobulin gene rearrangements; polymerase chain reaction; thyroid lymphoma
In the following report we discuss a very rare case of malignant T-cell lymphoma of the thyroid gland that developed in a 70-year-old woman with a past history of hypothyroidism due to chronic thyroiditis. The chief complaint was a rapidly growing neck mass. CT and ultrasonographic examination revealed a diffuse large thyroid gland without a nodule extending up to 13 cm. Although presence of abnormal lymphoid cells in the peripheral blood was not found, the sIL-2 Receptor antibody and thyroglobulin measured as high as 970 U/ml and 600 ng/mL respectively. Fine needle aspiration cytology diagnosed chronic thyroiditis. A preoperative diagnosis of suspicious malignant lymphoma of the thyroid gland accompanied by Hashimoto’s thyroiditis was made, and a right hemithyroidectomy was performed to definite diagnosis. Histological examination revealed diffuse small lymphocytic infiltration in the thyroid gland associated with Hashimoto’s thyroiditis. Immunohistochemical examination showed that the small lymphocytes were positive for T-cell markers with CD3 and CD45RO. The pathological diagnosis was chronic thyroiditis with atypical lymphocytes infiltration. However, Southern blot analysis of tumor specimens revealed only a monoclonal T-cell receptor gene rearrangement. Finally, peripheral T cell lymphoma was diagnosed. Therefore, the left hemithyroidectomy was also performed one month later. No adjuvant therapy was performed due to the tumor stage and its subtype. The patient is well with no recurrence or metastasis 22 months after the surgical removal of the thyroid. As malignant T-cell lymphoma of the thyroid gland with Hashimoto’s thyroiditis was difficult to diagnose, gene rearrangement examination needed to be performed concurrently.
Peripheral T-cell lymphoma; Thyroid; Hashimoto’s thyroiditis; Molecular diagnosis; Gene rearrangement
Primary thyroid mucosa-associated lymphoid tissue (MALT) lymphoma is a very rare subgroup of thyroid lymphoma, accounting for about 6 to 28% of all primary thyroid lymphomas. The purpose of this study was to evaluate its clinicopathological features and treatment outcomes.
We identified seven patients with thyroid MALT lymphoma who were treated between January 1997 and December 2007, and reviewed their clinicopathological features and follow-up outcomes.
There were five female and two male patients, and their mean age was 73 years. All patients presented with palpable neck mass. Two patients had hoarseness and dyspnea. All patients had a history of Hashimoto's thyroiditis with a mean of 175 months. Malignant lymphoma was suspected in only three patients using core needle biopsy. Four patients underwent thyroidectomy in the absence of preoperative pathologic confirmation, and histologic diagnosis was obtained after surgery. As initial treatment, complete surgical resection was performed in five patients, radiotherapy in one, and a combination of chemotherapy and radiotherapy in one. Six patients were alive for the mean follow-up period of 66 months and one patient died of unrelated causes. There were neither recurrences nor disease-specific mortalities.
When primary thyroid MALT lymphoma occurs in the thyroid or is confined to the neck, it responds well to local treatment such as surgical resection and external beam radiation therapy.
Primary thyroid MALT lymphoma; Hashimoto's thyroiditis; Diffuse large B-cell lymphoma
A case of primary low grade B cell lymphoma of the salivary gland associated with a low grade B cell lymphoma of the thymus and involvement of the skin is reported. The lesions in the salivary gland and in the thymus showed the typical features of a lymphoma arising from the mucosa associated lymphoid tissue (MALT) and comprised lymphatic follicles, centrocyte-like (CCL) cells and lymphoepithelial lesions. Immunohistochemistry and Southern blot analysis supported the hypothesis that these lesions can originate from the same cellular clone. These findings confirm the occurrence of low grade B cell MALT lymphoma in the thymus and the possibility of spread of MALT lymphoma to other mucosal sites.
Hashimoto's thyroiditis has been reported to be associated with many neoplastic and nonneoplastic thyroid pathologies. This retrospective study aims to determine the demographic profile of Hashimoto's thyroiditis in Sri Lankans, document ancillary pathologies in Hashimoto's thyroiditis, and determine whether there is an increased risk of occurrence of malignancies, benign neoplasms, and nonneoplastic benign lesions in Hashimoto's thyroiditis by comparing with thyroids showing multinodular goiters, follicular adenomas, and colloid nodules. The mean age of Hashimoto's thyroiditis is 43.3 years with the majority in the 41 to 60 year age group and a female to male ratio of 10.3 : 1. This study revealed a statistically significant increase of thyroid malignancies in association with Hashimoto's thyroiditis. The association of Papillary carcinoma, Non-Hodgkin's lymphoma, and Hurthle cell adenoma with Hashimoto's thyroiditis was statistically significant.
Primary gastrointestinal lymphoma comprises 10-15% of all non-Hodgkin lymphomas and encompasses 30-40% of the total extranodal lymphomas. Approximately 60-75% of cases occur in the stomach, and then the small bowel, ileum, cecum, colon and rectum. Lymphoid neoplasms may consist of mature B, T and less commonly extranodal NK/T cells. Of these, the two most frequently encountered histologic subtypes are extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), where Helicobacter pylori infection is implicated in a number of cases, and diffuse large B cell lymphoma. Several B cell lymphomas are associated with chromosomal aberrations. Enteropathy-associated T cell lymphoma, type I in particular, usually arises in a background of celiac disease. T cell gene rearrangement confirms clonality. NK/T cell neoplasms are invariably associated with Epstein-Barr virus infection and are often aggressive; thus, differentiation from a benign NK-cell enteropathy is paramount. Although incidence of other hematopoietic malignancies in the gastrointestinal tract such as plasma cell myeloma associated with amyloidosis, plasmablastic lymphoma, Hodgkin disease, histiocytic sarcoma and mast cell sarcoma is extremely rare, these entities have been documented, with the latter two demonstrating aggressive clinical behavior. Endoscopic ultrasonography is an important adjunct in disease staging and follow-up. Conservative antibiotic treatment of stage I MALT lymphomas with associated Helicobacter pylori infection achieves good clinical outcome with high remission rate. Chemotherapy, radiation and rarely surgery are reserved for advanced diseases or cases resistant to conservative therapy and those not associated with Helicobacter pylori infection.
Gastrointestinal lymphomas; MALT lymphoma; NK/T-cell enteropathy
Background: Gastric mucosa associated lymphoid tissue (MALT) lymphoma is a low grade B cell lymphoma histologically characterised by neoplastic B cells surrounding follicles in a marginal zone pattern and selectively infiltrating epithelium to form characteristic lymphoepithelial lesions.
Aims: To identify solitary epithelial cells in gastric MALT lymphoma and investigate their nature.
Methods: Anonymised endoscopic biopsies from eight B cell gastric MALT lymphomas and 10 control biopsies from chronic atrophic gastritis were selected. The numbers of solitary cytokeratin positive epithelial cells were assessed both semiquantitatively and quantitatively in immunostained sections. Chromogranin A expression was studied in sections consecutive to those stained for cytokeratin.
Results: Statistical analysis of the quantitative data confirmed that solitary epithelial cells were significantly more common in the lymphomas. The study of consecutive sections showed that the single cells express chromogranin A.
Conclusions: The presence of solitary, cytokeratin positive epithelial cells within the neoplastic infiltrate is a characteristic feature of gastric B cell lymphoma. These solitary epithelial cells are of neuroendocrine origin.
stomach; mucosa associated lymphoid tissue lymphoma; solitary epithelial cells
Primary cutaneous lymphomas are the second most common site of extranodal non-Hodgkin lymphoma. A specifically type named extranodal marginal zone B-cell lymphomas are indolent low-grade neoplasma.
We report a case of a 42-year-old white man with multiple subcutaneous tumors located on the trunk and neck. The histopathological exam showed a non-epidermotropic, dense lymphocytic infiltrate. Histologic, immunohistochemical and cytologenetic analysis diagnosed primary cutaneous B-cell lymphoma MALT type. Investigation for other extranodal MALT lymphoma gastrointestinal tract, lung, salivary and thyroid glands was negative. The patient refused radiotherapy, but he accepted every 6 months close follow-up. Over a seven years period, we noticed a progressively disappearance of the skin lesions.
The necessity of aggressive treatment of this disease with excellent prognosis is discussed.
The treatment necessity of primary cutaneous B-cell lymphoma MALT type is discussed.
primary cutaneous B-cell lymphoma; MALT type; therapy
Aim—To report the clinical and histological features and outcome of primary and secondary malignant lymphomas of the urinary bladder.
Methods—Eleven cases of malignant lymphoma of the urinary bladder were obtained from the registry of cases at St Bartholomews and the Royal London Hospitals. The lymphomas were classified on the basis of their morphology and immunophenotype, and the clinical records were reviewed.
Results—There were six primary lymphomas: three extranodal marginal zone lymphomas of mucosa associated lymphoid tissue (MALT) type and three diffuse large B cell lymphomas. Of the five secondary cases, four were diffuse large B cell lymphomas, one secondary to a systemic follicular follicle centre lymphoma, and one nodular sclerosis Hodgkins disease. Four patients with secondary lymphoma for whom follow up was available had died of disease within 13 months of diagnosis. Primary lymphomas followed a more indolent course. In one case, there was evidence of transformation from low grade MALT-type to diffuse large B cell lymphoma. The most common presenting symptom was haematuria. Cystoscopic appearances were of solid, sometimes necrotic tumours resembling transitional cell carcinoma, and in one case the tumours were multiple. These cases represented 0.2% of all bladder neoplasms.
Conclusions—Diffuse large B cell lymphoma and MALT-type lymphoma are the most common primary malignant lymphomas of the bladder. Lymphoepithelial lesions in MALT-type lymphoma involve transitional epithelium, and their presence in high grade lymphoma suggests a primary origin owing to transformation of low grade MALT-type lymphoma. Primary and secondary diffuse large B cell lymphomas of the bladder are histologically similar, but the prognosis of the former is favourable.
Key Words: bladder • lymphoma • mucosa associated lymphoid tissue lymphoma
Light and electron microscopic study of the thyroid gland and an enlarged cervical lymph node in a 75-year-old woman with Hashimoto's thyroiditis disclosed immunoblastic proliferation in the lymph node, marked by collections of striking round cells positive to periodic acid-Schiff (PAS) staining, immunoblasts and plasmacytoid elements in a vascular, fibrous framework. The PAS-positive cells ("macaroni cells") were distended with whorls of angulated tubular material resembling endoplasmic reticulum. Parafollicular-cell hyperplasia and an atypical plasmacytoid focus were noted in the thyroid. Hashimoto's disease is known to be associated with malignant lymphoma, as are autoimmune and malignant diseases with immunoblastic lymphadenopathy. This is the first report of the association of Hashimoto's disease and immunoblastic lymphadenopathy. The atypical plasma cells have not previously been described.
Gastric B-cell lymphoma of the mucosa associated lymphoid tissue (MALT) lymphoma is one of the most common forms of extranodal lymphoma. In addition to infection with Helicobacter pylori (H. pylori), the presence of an underlying autoimmune disease has also been associated with MALT lymphoma development. To date, no familial predisposition for MALT lymphomas has been reported as opposed to other types of lymphoma. A 65-year-old woman was admitted at our institution in 1998 with a diagnosis of H. pylori positive gastric MALT lymphoma and the presence of chronic autoimmune thyroiditis was established on further work-up. H. pylori eradication did not result in regression of the lymphoma and RT-PCR showed the presence of the t(11;18)(q21;q21) translocation. About 1.5 years after H. pylori eradication, chemotherapy with cladribine resulted in complete remission. Due to lymphoma recurrence 13 mo later, radiotherapy to the stomach (46 Gy) resulted in minimal residual disease without further progression. The patient developed a second malignancy (Epstein-Bar virus-associated anaplastic large cell lymphoma in the mediastinum) in 2004 which initially responded to two courses of chemotherapy, but she refused further therapy and died of progressive lymphoma in 2006. In 2008, her 55 years old daughter with a long standing Sjögren’s syndrome was diagnosed with MALT lymphoma of the right parotid, but no evidence of gastric involvement or H. pylori infection was found. Currently, she is alive without therapy and undergoing regular check-ups. To our knowledge, this is the first report of MALT lymphoma in a first-degree relative of a patient with gastric MALT lymphoma in the context of two autoimmune diseases without a clearly established familial background.
Mucosa associated lymphoid tissue lymphoma; Helicobacter pylori; Autoimmunity; Familial lymphoma
Lymphoma of the salivary gland accounts for 5% of cases of extranodal lymphoma and 10% of malignant salivary gland tumours. Most primary salivary gland lymphomas are B marginal zone lymphomas arising on a background of sialadenitis associated with autoimmune disorders such as Sjorgen's syndrome. Primary T cell lymphoma of the salivary gland is rare. This report describes a case of primary T cell lymphoma arising in the parotid gland of an elderly white man, which was notable for its striking resemblance to a B cell extranodal marginal zone lymphoma. Immunohistochemistry and gene rearrangement studies confirmed the clonal T cell nature of the tumour. There was no molecular evidence of Epstein-Barr virus (EBV) infection of neoplastic or surroundings cells. Only 14 cases of primary T cell lymphoma of the salivary glands have been recorded in the literature, most being from the Orient and having extremely variable prognosis. Those with a T/natural killer cell phenotype are associated with EBV infection. This case highlights the fact that T cell lymphoma in the salivary gland can mimic closely the morphological features of B cell extranodal marginal zone lymphoma.
salivary gland; T cell lymphoma; Epstein Barr virus
Hashimoto's thyroiditis (HT), an autoimmune disorder, is the most prevalent cause of subclinical or overt hypothyroidism in areas with sufficient iodine intake. The gland is often diffusely enlarged, and the parenchyma is coarsened, hypoechoic, and often hypervascular on ultrasonograpy. Histopathologic appearance of HT includes lymphocyte aggregates with germinal centers, small thyroid follicles, presence of Hurthle cells, and variable fibrosis. We present a case of a 40-year-old female with suspected follicular neoplasm on fine-needle aspiration cytology of neck swelling. Intraoperatively, thyroid gland was found having four lobes separated from each other. Total thyroidectomy was done and histopathology from all four lobes revealed HT. At present, there is no literature to support the fact that such distorted thyroid anatomy may be due to the underlying disease. If we consider it as thyroid gland anomaly, no such anomaly has been mentioned in the literature till date.
Four lobed thyroid gland; Hashimoto's thyroiditis; Thyroid anomaly; Thyroidectomy
Mucosa-associated lymphoid tissue (MALT) is a type of extra nodal malignant lymphoma seen in organs such as the stomach, thyroid and salivary glands. Furthermore, occurrence of colorectal MALT lymphoma is extremely rare. We report a case of a solitary rectal MALT lymphoma treated by surgical resection and radiotherapy. Lymphoma should be considered as a rare differential diagnosis when dealing with large bowel pathology. We would advocate the use of surgery as a primary treatment option for a medically fit patient.
Mucosa associated lymphoid tissue lymphomas are the most common lymphomas of the salivary glands. The benign lymphoepithelial lesion is also a lymphoproliferative disease that develops in the parotid gland. In the present case report, we describe one case of benign lymphoepithelial lesion with a subsequent low transformation to grade mucosa associated lymphoid tissue lymphoma appearing as a cystic mass in the parotid gland.
A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision. The pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (mucosa associated lymphoid tissue lymphoma) following benign lymphoepithelial lesion of the gland.
Salivary gland mucosa associated lymphoid tissue lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. Parotidectomy is recommended in order to treat the tumor and to ensure histological diagnosis for further follow-up planning. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal.
Primary duodenal mucosa associated lymphoid tissue (MALT) lymphoma is very rare, and little is known about its clinical course or effective treatment. We describe a case of primary duodenal MALT lymphoma that was resistant to Helicobacter pylori (H. pylori) eradication and regressed after chemotherapy with cyclophosphamide, vincristine, and prednisolone (CVP). A 71-year-old woman was referred to our department because of epigastric pain and dyspepsia. Gastroduodenoscopy revealed an irregular mucosal nodular lesion with ulceration extending from the bulb to the second portion of the duodenum. Histopathological examination of a biopsy specimen disclosed low-grade MALT lymphoma composed of atypical lymphoid cells with lymphoepithelial lesion. Abdominal CT scans revealed 0.5 to 1.5 cm lymph nodes in the peritoneal cavity, suggestive of lymph node metastasis. We successfully eradicated H. pylori but did not see signs of remission. We administered systemic CVP chemotherapy every 3 weeks. After 6 courses of CVP, the patient achieved complete remission and was followed up without recurrence for about a year.
MALT lymphoma; Duodenum; Helicobacter pylori; Chemotherapy
The risk of misdiagnosing neoplastic cells typically infiltrating an epithelium forming a lymphoepithelial lesion as poorly differentiated gastric cancer in endoscopic biopsies, particularly in low-grade mucosa-associated lymphoid tissue (MALT) lymphomas, is described. A 76-year-old woman was referred for management of a poorly differentiated gastric adenocarcinoma. Diagnostic endoscopy in our unit showed a 2 cm raised, submucosal lesion with central erosion in the upper body of the stomach, but repeat biopsies of the lesion were interpreted as inflamed gastric mucosa and negative for malignancy. Systematic gastric biopsies to rule out any foci of MALT lymphoma changes were also negative. Therefore, endoscopic submucosal dissection was performed to obtain an accurate specimen. Histology revealed centrocyte-like cells and a lymphoepithelial lesion invading into the mucosa with obliteration of the gastric glands, which was initially interpreted as poorly differentiated adenocarcinoma.
Mucosa-associated lymphoid tissue lymphoma; Poorly differentiated gastric adenocarcinoma; Centrocyte-like cell; Lymphoepithelial lesion
Immunoproliferative small intestinal disease (originally called Mediterranean lymphoma and subsequently alpha chain disease) is a slowly progressive low grade primary small intestinal B-cell lymphoma characterized by the synthesis of a truncated alpha heavy chain without light chain by the neoplastic cells. The histological features of IPSID and low grade primary gastric B-cell lymphoma are closely similar and recapitulate the those of Peyer's patches. This observation has led to the mucosa associated lymphoid tissue (MALT) lymphoma concept which encompasses a group of extranodal lymphomas including IPSID and primary gastric lymphoma. Unlike nodal low-grade B-cell lymphomas, IPSID and low grade gastric lymphoma remain localized to their sites of origin for prolonged periods. One possible explanation for this is that the growth of these lymphomas is influenced by a local antigen. This is supported by reports of clinical remissions induced in IPSID following sterilization of the small intestine using broad spectrum antibiotics. Similar findings have been reported in low grade gastric lymphoma following eradication of Helicobacter pylori which is almost invariably present in the patients' stomachs. Laboratory experiments have shown that the growth of lymphomatous B-cells is stimulated via H. pylori specific T-cells. Further work is required to identify the antigen(s) operative in IPSID and, possibly, other low grade B-cell lymphomas.