Surgical management of pulmonary mucosa-associated lymphoid tissue-derived lymphoma: retrospective study of 86 cases / 中华胸心血管外科杂志

Objective:To summarize our experience in diagnosis and treatment of patients with pulmonary mucosa-associated lymphoid tissue-derived(MALT) lymphoma and to explore the role of surgery.Methods:We retrospectively analyzed the clinical and follow-up data of 86 patients with pulmonary MALT lymphoma in Shanghai Pulmonary Hospital from January 2000 to December 2018. 86 cases were identified with 44 males and 42 females. The mean age was(56.7±10.6) years old. 38(44.2%) cases had symptoms mainly presenting as cough and sputum at diagnosis. The chest CT scan of the patients showed pulmonary consolidation in 39 cases, nodule/mass shadow in 37 cases, usually with air bronchogram. Only 8(9.3%) cases could be diagnosed by non-surgical approach. 58 cases received complete resection, 10 of which followed chemotherapy. 8 of 17 cases who received incomplete resection accepted chemotherapy. The rest 11 cases who experienced surgical biopsy because of uncertain diagnosis were treated by chemotherapy or radio-chemotherapy or just watch-to-wait.Results:The median follow-up of 83 cases was 64 months, ranged from 24 to 219 months. The estimated 5-year and 10-year OS rates were 95.0% and 76.8%, while 5-year and 10-year PFS were 75.7% and 35.1% respectively. Patients who received complete resectionhad better PFS( P<0.001)but similar OS( P=0.395), compared with those received incomplete resection. There were no significant difference in OS and PFS between patients who received complete resection accepted chemotherapy or not( P>0.05). Conclusion:Pulmonary MALT lymphoma has an indolent nature with an excellent long-term survival. Diagnosis is difficult to be made by non-surgical approach. Surgery plays an important role of treatment of pulmonary MALT lymphoma, due to significant improvement of diagnosis rate and radical treatment of localized disease by complete resection.

Linfoma no Hodgkin tipo MALT cerebral / Cerebral type MALT non Hodgkin lymphoma

Introducción: Los linfomas no Hodgkin tienen su origen generalmente en el sistema linfoide, existe una gran diversidad de ellos, que hace difícil su clasificación y diagnóstico anatomopatológico definitivo. También hay localizaciones extranodales, como en el cerebro, lo que la hace aún más interesante. Objetivo: Presentar un caso con linfoma marginal cerebral, de diagnóstico muy infrecuente. Caso clínico: Paciente femenina de 67 años de edad con antecedentes patológicos personales de hipertensión arterial y epilepsia, que comenzó a sentirse rígida, con movimientos involuntarios generalizados en el cuerpo, hasta perder la conciencia. Al recuperarse se encontraba somnolienta y con cefalea de moderada intensidad. Se diagnostica un linfoma no Hodgkin tipo MALT cerebral; se realiza tratamiento con el que se revierten los síntomas y se controla la enfermedad. Conclusiones: Es necesario un diagnóstico histopatológico oportuno y certero, con la utilización de los estudios inmunohistoquímicos. Esto permite aplicar un tratamiento precoz y el control de la enfermedad(AU)

Introduction: Non-Hodgkin's lymphomas generally have their origin in the lymphoid system, there is a great diversity of them, which makes their classification and definitive pathological diagnosis difficult. There are also extranodal locations, such as in the brain, which makes it even more interesting. Objective: To present a patient with marginal cerebral lymphoma, of a very infrequent diagnosis. Clinical case: A 67-year-old female patient with a personal pathological history of arterial hypertension and epilepsy, who began to feel rigid, with involuntary movements that generalized to the whole body, until she lost consciousness, when she recovered she was drowsy and with headache of moderate intensity. Brain MALT non-Hodgkin lymphoma is diagnosed, treatment is performed to reverse symptoms, and the disease is controlled. Conclusions: A timely and accurate histopathological diagnosis is necessary, with the use of immunohistochemical studies. This allows for early treatment and disease control(AU)

Linfoma no Hodgkin tipo MALT de la glándula parótida. Presentación de un caso / MALT non-Hodgkin Lymphoma of the Parotid Gland. Presentation of a Case

Resumen Los linfomas de las glándulas salivales son una entidad e incidencia rara. La localización más frecuente es la glándula parótida, seguida de las glándulas submandibular y sublingual. La mayoría de los linfomas parótidos son linfomas no Hodgkin y se consideran derivados de tejido linfoide asociado a la mucosa (MALT). De manera poco frecuente, estos casos se han notificado y con frecuencia se diagnostican de manera inadecuada, ya que su presentación es relativamente benigna, comportamiento localizado, crecimiento lento y varios años de evolución.

Abstract Lymphomas of the salivary glands are a rare entity with a rare incidence. They are more commonly found on the parotid gland, followed by the submandibular and sublingual glands. The majority of parotid lymphomas are non-Hodgkin's lymphomas and are considered to be derived from mucosal-associated lymphoid tissue (MALT). Reports of these cases are scarce and often misdiagnosed since: their manifestation is relatively benign, they have a localized behavior, a slow growth and the evolution takes several years.

Analysis of clinical and prognostic features in 180 patients with mucosa associated lymphoma tissue lymphoma / 中国肿瘤临床

Objective: A single-center retrospective analysis was performed to obtain data on the clinical characteristics and the factors influencing the survival of patients with mucosa associated lymphoma tissue lymphoma (MALT lymphoma). Method: From September 2012 to May 2019, we retrospectively analyzed 180 cases of MALT lymphoma diagnosed through pathological biopsy. Result: Among the 180 patients, 99 (55.0%) were male and 81 (45.0%) were female. The age at onset was 31-84 years, and the median age was 57 years. There were 59 (32.8%), 66 (96.1%), and 55 (3.9%) cases involving gastrointestinal, ocular, and other extranodal primary sites, which included the lung, pharyngeal lymph ring, thyroid, parotid gland, and testis, respectively. Moreover, there were 173 (94.4%) cases of clinical stage -Ⅱdisease and 7 cases (5.6%) of clinical stage III- disease. The 5-year overall survival (OS) rates in the gastrointestinal, ocular, and other groups were 96.7%, 95.5%, and 86.4%, respectively, and the difference was not statistically significant (P= 0.118). The 5-year progression-free survival (PFS) rates were 82.4%, 86.6%, and 86.4% in the corresponding groups, respectively, and the difference was not statistically significant (P=0.862). The single-factor correlation analysis of prognosis indicated that low hemoglobin (P=0.006) and high β2-microglobulin (P=0.013) levels were related to OS, while an increase in β2-microglobulin (β2-MG) (P=0.004) levels was related to OS and PFS. Survival analysis showed that a low hemoglobin level was significantly associated with OS (P=0.015). Conclusions: MALT lymphoma can originate from different parts of the body. Additionally, the patients show slow clinical progress, late recurrence, and long total survival. Low hemoglobin and β2-MG levels can be used as important indexes for clinical prognosis.

Adenocarcinoma pulmonar sincrónico y linfoma primario de tejido linfoide asociado a mucosa pulmonar / Synchronous pulmonary adenocarcinoma and primary lymphoma of lung mucosa-associated lymphoid tissue

Los linfomas derivados del tejido linfoide asociado a las mucosas (MALT) son entidades poco frecuentes, de bajo grado de malignidad con escaso o nulo compromiso ganglionar y representan cerca del 80% de los linfomas primarios pulmonares. La aparición sincrónica con adenocarcinoma de pulmón es un hallazgo extremadamente infrecuente. Presentamos el caso de un hombre de 68 años, ex-tabaquista, en quien durante el seguimiento de un nódulo pulmonar se identificó un segundo nódulo y la biopsia quirúrgica confirmó el diagnóstico de ambas neoplasias.

The lymphomas of mucosa-associated lymphoid tissue (MALT), are uncommon entities, of low grade of malignancy with very infrequent or no lymph node involvement. They represent about 80% of the primary pulmonary lymphomas. The synchronous appearance with lung adenocarcinoma is an extremely rare finding. We present the case of an ex-smoker 68-year-old man, in whom, in the follow-up of a pulmonary nodule, a second pulmonary nodule was found. The surgical biopsy confirmed the diagnosis of both neoplasms.

Primary thyroid lymphoma: A series from a tertiary care center in Northern India

Objective: Primary thyroid lymphoma (PTL) is a rare entity, necessitating accurate and early diagnosis, as its management is very different from that of other neoplasms intrinsic to the thyroid. Materials and Methods: Cases diagnosed between January 2009 and March 2015 were retrieved, and clinical details were noted. Hematoxylin- and eosin-stained slides were reviewed. Immunohistochemistry (IHC) was performed for immunophenotyping, and cases were classified according to the World Health Organization 2017 classification of hematolymphoid neoplasms. Results: Eleven patients with PTL were identified, with a mean age of 64.6 years (range: 40–76 years), including three males and eight females. Duration of symptoms ranged from 2 to 36 months (mean: 9.3 months). Diffuse large B-cell lymphoma (DLBCL) was most frequent, followed by extranodal marginal zone lymphoma. Most DLBCLs were nongerminal center type. BCL2 was positive in all DLBCLs. Strong p53 immunopositivity was not seen in any of the cases analyzed. Conclusion: Histopathological evaluation supplemented by IHC is the gold standard for the diagnosis of PTL. Combined chemoradiotherapy appears to be the best treatment modality, irrespective of histological type. MIB-1 and MUM1 IHC may have a role in identifying DLBCL, particularly in small biopsies. Role of p53 and BCL2 needs further evaluation

A Case report of MALT lymphoma in parotid gland / 대한두경부종양학회지

Mucosa-associated lymphoid tissue (MALT) lymphoma has specific clinical and pathologic features. The most common site MALT lymphomas is the stomach; however, it can also occur in other organs, such as the salivary glands. MALT lymphoma is rare, but its prognosis is good. A 32-year-old man visited Konyang university hospital with parotid mass. Superficial partial parotidectomy was performed to exclude lymphoid neoplasms. IgH gene rearrangement analysis of the surgical specimen led to the diagnosis of MALT lymphoma. The patient underwent esophagogastroduodenoscopy, positron emission tomography-computed tomography, and whole-body bone scan. Regional or distant metastasis was not observed on staging workup. The patient underwent postoperative radiation therapy, there has been no recurrence of MALT lymphoma to date. Here, we report this rare case of parotid MALT lymphoma that was treated with surgery and postoperative radiation therapy.

Primary Lymphoma of Pancreatic Mucosa-Associated Lymphoid Tissue (MALT) / 대한내과학회지

Primary lymphoma of pancreatic mucosa-associated lymphoid tissue (MALT) is extremely rare. Initial suspicion of primary pancreatic lymphoma is hampered by its low incidence. However, it should always be included in a differential diagnosis of pancreatic mass with unusual features, because an accurate diagnosis can avoid unnecessary surgical intervention. A 70-year-old woman presented with melena associated with a mass on the pancreatic head. Endoscopic ultrasonography-guided core-needle biopsy of the pancreatic mass revealed MALT lymphoma. The patient is currently undergoing radiation therapy. We present a case of primary lymphoma of the pancreatic MALT with a review of the literature.

Expression of integrin-linked kinase (ILK) and Toll-like receptor (TLR-2) in ocular adnexal MALT lymphoma and the effects on patients' prognosis / 眼科新进展

Objective To investigate the expression of integrin-linked kinase (ILK) and Toll-like receptor (TLR-2) in ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma and the effects on patients' outcomes.Methods Tissue specimens from 46 patients with MALT lymphoma were collected in this study.The expression of ILK and TLR-2 protein was detected by immunohistochemical methods,and the correlation of ILK and TLR-2 protein expression with clinicopathological features and patients' outcomes was analyzed.Results The positive rates of ILK and TLR-2 protein expression in tumor tissues were 67.4％ (31/46) and 71.7％ (33/46),respectively,which was related to the clinical stage of AnnArbor (P ＜ 0.05),rather than to sex,age and lesion location (all P ＞0.05).The survival of patients with ILK positive expression was less than that of ones with ILK negative expression [(21.5 ± 2.7)months vs.(29.2 ± 2.1) months] (P ＜ 0.05);meanwhile,the survival of patients with TLR-2 positive expression was less than that of ones with TLR-2 negative expression [(20.4 ±1.7) months v.(27.6 ± 2.3) months] (P ＜ 0.05).Conclusion ILK and TLR-2 are closely related to biological behavior of ocular adnexal MALT lymphoma,and combination detection of ILK and TLR-2 has a certain guiding value for diagnosis and prognosis.

Primary Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue of the Lacrimal Sac Mimicking Dacryocystitis

PURPOSE: We report a case of a young female patient who was diagnosed with a marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) of the lacrimal sac which mimicked dacrocystitis. CASE SUMMARY: A 23-year-old female suffered from epiphora for 3 years in the right eye. She had swelling and a painful lesion at the lacrimal sac 4 months prior and was referred to our hospital due to nasolacrimal duct obstruction and dacryocystitis. Lacrimal irrigation was performed with no passing and regurgitation with mucoid discharge. We performed orbital computed tomography and magnetic resonance imaging which showed a suspected tumor of the lacrimal sac in the right eye. We then performed excision and biopsy of the tumor through the skin approach. The patient was diagnosed with a MALT lymphoma with no systemic involvement. After six cycles of chemotherapy involving rituximab with cyclophosphamide, vincristine, and prednisone, the lesion of the mass and the painful symptoms decreased, which was regarded as a complete response. However, epiphora and eye discharge persisted, showing a nasolacrimal duct obstruction, so we performed endoscopic dacryocystorhinostomy with a lacrimal sac biopsy. The histological examination showed chronic inflammation, but not lymphoma. There was no recurrence of lymphoma at one year postoperatively. CONCLUSIONS: If there is an epiphora and palpable lesion in the lacrimal sac area, MALT lymphoma mimicking dacyocystitis may be suspected. After the lymphoma is first diagnosed by excision and biopsy, systemic chemotherapy with dacryocystorhinostomy could be an effective treatment.

Benefit of volumetric-modulated arc therapy over three-dimensional conformal radiotherapy for stage I-II extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue in the stomach: a dosimetric comparison

PURPOSE: To retrospectively analyze dosimetric parameters of volumetric-modulated arc therapy (VMAT) and three-dimensional conformal radiotherapy (3D-CRT) delivered to extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue in the stomach (gastric MALT lymphoma) to find out advantages of VMAT and conditions for definite benefits of VMAT. MATERIALS AND METHODS: Fifty patients with stage I-II gastric MALT lymphoma received VMAT (n = 14) or 3D-CRT (n = 36) between December 2005 and April 2018. Twenty-seven patients were categorized according to whether the planning target volume (PTV) overlaps kidney(s). Dosimetric parameters were analyzed by dose-volume histogram. RESULTS: Radiation dose to the liver was definitely lower with VMAT in terms of mean dose (p = 0.026) and V15 (p = 0.008). The V15 of the left kidney was lower with VMAT (p = 0.065). For those with PTV overlapping kidney(s), the left kidney V15 was significantly lower with VMAT. Furthermore, the closer the distance between the PTV and kidneys, the less the left kidney V15 with VMAT (p = 0.037). Delineation of kidney(s) by integrating all respiratory phases had no additional benefit. CONCLUSIONS: VMAT significantly increased monitor units, reduced treatment time and radiation dose to the liver and kidneys. The benefit of VMAT was definite in reducing the left kidney V15, especially in geometrically challenging conditions of overlap or close separation between PTV and kidney(s).

Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand

BACKGROUND: Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand. METHODS: We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes. RESULTS: Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22–86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01). CONCLUSION: We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.

Clinical, endoscopic, pathologic and prognostic factors of primary gastric lymphoma / 中华消化杂志

Objective To explore the clinical,endoscopic,pathologic and prognostic characteristics of primary gastric lymphoma (PGL) and to improve the level of diagnosis and treatment.Methods Sixtythree patients who were confirmed as PGL with operation and endoscopic biopsy pathology during January 2001 to December 2010 were retrospectively analyzed with respects of clinical,endoscopic and pathologic features.Survival analysis and prognosis were evaluated by kaplan-Meier and Cox proportional hazard model,respectively.Results In 63 PGL patients,the numbers of male and female were 40 and 23,respectively,and the average age was (59.8±13.3)years.The major symptoms were abdominal pain,abdominal distension,and gastrointestinal hemorrhage,accounting for 47.6 ％ (30/63),17.5 ％ (11/63),and 17.5 ％ (11/63),respectively.There were 39 (61.9 ％) PGL patients with endoscopic performance for ulcers,34 (54.0 ％) cases involved the gastric stomach antrum.The most immunohistochemistry analyses were diffuse large B-cell lymphoma (DLBCL) (71.4 ％,45/63),followed by mucosa-associated lymphoid tissue (MALT) lymphoma (22.2％,14/63).The frequency of Helicobacter pylori (H.pylori) positivity was lower in patients with DLBCL than that in patients with MALT lymphoma (37.8％(17/45) vs 10/14,x2 =4.872,P=0.027).The accumulate survival rates of one,three and five years were 74.6％,63.5％,55.6％,respectively,and the average survival time was (41.5±3.0) months (95％ confidence interval (CI) 35.7 to 47.4 months) in PGL patients.There was no difference in the average survival time between DLBCL patients treated with surgery combined chemotherapy and those with surgery or chemotherapy alone (38.33±5.21) months vs (50.17±8.98) months vs (41.39±4.40) months,P＞0.05).The patients diagnosed as DLBCL with H.pylori positive had longer average survival time than those with H.pylori negative ((51.90±4.30) months vs (33.30±4.50) months,t=-4.004,P＜0.01).Conclusions Male patients with PGL are slightly more than female.Abdominal pain is the most frequent symptom.Ulcerative lesions are the most common endoscopic demonstrations mostly at stomach sinus.DLBCL is the most pathologic characteristic.There is no significant difference in the survival rate between patients treated with surgery combined with chemotherapy and those treated with surgery or chemotherapy alone.

Clinicopathological Characteristics of Primary Renal Mucosa Associated Lymphoid Tissue Lymphoma:A Case Report and Review Literature / 现代检验医学杂志

Objective Primary renal mucosa associated lymphoid tissue lymphoma (MALT lymphoma)is very rare,here dis-cuss the clinical pathological characteristics and diagnosis,differential diagnosis of primary renal MALT lymphoma.Methods A case of primary renal MALT lymphoma was assayed for clinical and histopathological morphology,an well as immuno-histochemical staining,then reviewed the relevant literature.Results The clinical and imaging characteristics of renal lym-phoma lack of specific performance,the tissue section of the kidney biopsy showed diffuse infiltration of small lymphocytes. The small lymphocytes were centrocytes-like,monocytes-like,and plasma cell differentiation was recognized.Lymphoepithe-lial lesions were scattered.Follicular implanted phenomenon could been seen.Immunohistochemically result:the tumor cells were positive for CD20,PAX-5,CD79αand MUM-1,but negative for CD3,CD5,CD45RO,bcl-2,CD10,bcl-6 and CyclinD1. CD2 1 showed damage FDC network,positive for Kappa,negative for lambda,and it showed monoclonal phenotype,the Ki-6 7 index was about 35%.Final pathology diagnosis was primary renal MALT lymphoma.Conclusion Primary renal MALT lymphoma is very rare,the clinical manifestations and imaging characteristic of renal lymphoma are non-specific and misdiag-nosis frequently occurs in clinical practice,definite diagnosis depends on pathological examination,immunohistochemical stai-ning is helpful to differential diagnosis.

Mucosa-Associated Lymphoid-Tissue Lymphoma of the Cecum and Rectum: A Case Report

A colonic mucosa-associated lymphoid-tissue (MALT) lymphoma is relatively rare compared to lymphomas of the stomach or small intestine. We present a case of a MALT lymphoma in the cecum and rectum found during screening colonoscopy. A 54-year-old female, who had undergone right-breast-conserving surgery with axillary dissection due to an invasive ductal carcinoma and a left-breast excisional biopsy due to microcalcification following adjuvant chemoradiation therapy 3 years earlier, was found to have 3-mm-sized smooth elevated lesions in both the cecum and rectum. No pathologic lesion or lymphadenopathy was found at any other site, but chronic gastritis negative for Helicobacter pylori infection was found. The polyps were removed by using an endoscopic biopsy and revealed an extra nodal marginal zone B-cell MALT lymphoma, showing positive for CD3 and CD20 by immunohistochemical staining. The patient underwent close observation without any additional treatment and has shown no evidence of recurrence as of her last visit.

Linfoma ocular de células B da zona marginal: relato de caso e revisão da literatura / Ocular B-cell marginal zone lymphoma: case report and literature review

RESUMO Paciente feminina, 70 anos, apresentou-se com queixas de prurido ocular à esquerda, lacrimejamento e hiperemia há 2 meses. Ao exame físico, apenas hiperemia conjuntival discreta. Foi solicitada biópsia, que sugeriu a hipótese de pseudolinfoma pela insuficiência de critérios classificatórios. Após um ano a paciente retornou com os mesmos sintomas e proptose ocular à esquerda, associado a proliferação de aspecto linfático em conjuntiva bulbar, com presença de vasos sanguíneos, ocupando toda região superior e maior parte da medial, se estendendo até o limbo, sem oclusão do eixo visual e mobilidade ocular extrínseca preservada. Nova biópsia e imuno-histoquímica, foram compatíveis com linfoma de células B da zona marginal. Foram realizadas tomografias computadorizadas (TC) de crânio, pescoço e órbitas, revelando aumento do volume do músculo reto superior esquerdo, com intensa impregnação pelo contraste, acometendo ventre e tendão, com borramento de gordura adjacente. Foram programados 6 ciclos de quimioterapia com ciclofosfamida, doxorrubicina, vincristina e prednisona, CHOP, a cada 21 dias. Houve melhora dos sintomas oculares após o primeiro ciclo. Após o quarto, nova TC de órbitas evidenciou regressão do espessamento do músculo reto superior esquerdo, ainda melhor na TC realizada após o sexto ciclo. Os linfomas MALT são responsáveis por 5-17% de todos os casos de LNH, respondem por mais de 90% dos linfomas que acometem os anexos oculares, mas podem se originar em diversos tecidos. Devido a raridade da doença, carecem estudos prospectivos randomizados que definam um consenso terapêutico. A literatura sugere que o tratamento deve ser individualizado.

ABSTRACT Female patient, 70 years old, presented with complaints of left eye pruritus, tearing and redness, for 2 months. Physical examination revealed only mild conjunctival hyperemia. Biopsy was ordered, and suggested the hypothesis pseudo lymphoma by insufficient classification criteria.After a year, the patient returned with the same symptoms, and left ocular proptosis associated with lymphatic aspect of proliferation in bulbar conjunctiva, with the presence of blood vessels, occupying the entire upper region and most of the medial, extending into the limbo without occlusion the visual axis, extrinsic ocular motility preserved.Another biopsy and immunohistochemistry were compatible with a B cell marginal zone lymphoma. Tomographic studies of cranium, neck and orbits were performed, and the orbit showed enlargement of the left superior rectus muscle volume, with intense contrast uptake, affecting belly and tendon and blurring of adjacent fat. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone, CHOP, were done every 21 days. There was improvement in ocular symptoms after the first cycle. After the fourth, new scans from the orbits revealed regression of thickening of the left superior rectus muscle, and even better after the sixth cycle. The MALT lymphomas account for 5-17% of all cases of NHL, accounting for over 90% of lymphomas affecting the eye attachments, but can originate in different tissues. Due to the rarity of the disease, there are no randomized prospective studies to define a therapeutic consensus. The literature suggests that treatment should be individualized.

The Hidden Salmon Patch: Ocular Lymphoma Mistaken as Vogt Koyanagi Disease

Ocular inflammation from various causes may have similar clinical presentation thus careful clinical evaluations are mandatory particularly when the disease appear to be resistant to treatment. This paper reports a case of ocular lymphoma which was initially treated as Vogt Koyanagi Harada disease at a different centre. A 65-year-old Sudanese man complained of gradual worsening left eye vision. Careful ocular and slit lamp examination revealed a conjunctival lesion with choroidal infiltration as well as exudative retinal detachment. Computed tomography scan (CT scan) showed left eye axial proptosis, and a homogenous enhancing mass at the posterior coat of the globe. Tissue biopsy of the conjunctival lesion revealed marginal Zone B cell (MALT type), low grade, non-Hodgkin’s lymphoma. AS the ocular signs and symptoms progressed, chemotherapy was initiated. The proptosis, exudative retinal detachment, disc hyperemia and swelling improved after chemotherapy. The post treatment CT scan showed reduction of the posterior ocular coat mass. The possibility of malignant lymphoma should be considered in patients with resistant uveitis despite administration of corticosteroid. Ocular lymphoma is an indolent tumour with good prognosis. Careful ocular examination, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis.

Clinicopathologic study of primary thymic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue and lymphoepithelial sialadenitis-like thymic hyperplasia / 临床与实验病理学杂志

Purpose To discuss the clinicopathological features of primary thymic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT) lymphoma and lymphoepithelial sialadenitis (LESA)-like thymic hyperplasia,their relationship and differential diagnosis.Methods Three cases of thymic MALT lymphoma and one LESA-like thymic hyperplasia were evaluated by HE staining,immunohistochemistry and immunoglobulin (Ig) gene rearrangement technology.Results The symptoms of Sjsgren syndrome were found in the two patients with thymic MALT lymphoma.Microscopically,the normal architecture of thymus was effaced by dense lymphoid infiltration composed predominantly of centrocyte-like and monocytoid B cells with prominent lymphoepithelial lesions.Immunohistochemically,the tumor cells were positive CD20,PAX-5,and BCL-2.The plasma cells showed lambda light chain restriction in one case with prominent plasmacytoid differentiation.In LESA-like thymic hyperplasia,the normal lobular architecture of thymus was generally reserved and abundant lymphoid tissue containing lymphoid follicles was seen with prominent lymphoepithelial lesions in expanding islands of thymic epithelial cells and epithelium lining the cysts,but a monocytoid B-cell population was absent.Immunohistochemically,a mixed B-cell and T-cell population was identified.A monoclonal rearrangement of the Ig gene was detected in all three thymic MALT lymphomas but not in the case of LESA-like hyperplasia.Conclusion Primary thymic MALT lymphoma and LESA-like thymic hyperplasia are both rare lymphoid proliferative lesions and the two lesions have overlapping histological and immunohistochemical features.A combination of genetic rearrangement and analysis of the differential points is helpful to distinguish between them.

A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome

PURPOSE: To report a case of lacrimal gland mucosa-associated lymphoid tissue (MALT) lymphoma in a patient with primary Sjögren's syndrome and Behcet's disease. CASE SUMMARY: A 49-year-old female patient with primary Sjögren's syndrome and Behcet's disease presented with a one-year history of painless upper and lower eyelid swelling in her right eye. Lacrimal gland incisional biopsy was performed, and the patient was diagnosed with malignant lymphoma (extranodal marginal zone B cell lymphoma of MALT). No distant metastases were detected on whole-body computed tomography or positron emission tomography, and the patient was treated with Rituximab, Cyclophosphamide, Vincristine, Prednisone (R-CVP) regimen chemotherapy. After 8 consecutive chemotherapy cycles, her eyelids appeared normal externally, and partial regression was found radiologically. CONCLUSIONS: The possibility of MALT lymphoma should be considered as a differential diagnosis if patients with autoimmune diseases such as primary Sjögren's syndrome show eyelid swelling or palpable mass.

Diffuse Large B-Cell Lymphoma Transformed from a Rectal Mucosa-Associated Lymphoid Tissue Lymphoma / 이화의대지

Primary rectal lymphoma is a rare disease among the gastrointestinal (GI) lymphoma. In particular, diffuse large B-cell lymphoma (DLBCL) transformed from mucosa-associated lymphoid tissue (MALT) lymphoma is often the primary type of GI lymphoma, mostly in stomach or duodenum, but has never been reported in rectum. Here we report an unusual case in which a 75-year-old male patient diagnosed with DLBCL transformed from MALT lymphoma in the rectum. The patient was diagnosed as rectal DLBCL transformed from MALT lymphoma as Lugano stage II2 and was treated with chemotherapy (R-CHOP) with CD-20 monoclonal antibody (rituxaimb). Complete remission of multiple lymphadenopathy and mass forming ulcer of the rectum was achieved after 6 cycles of R-CHOP. He has been free from disease for 12 months.