Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center / 中华血液学杂志

Objective: This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . Methods: Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. Results: A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. Conclusions: Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.

Histomorphological structure of the Bursa cloacalis (Bursa of Fabricius) in young Leiothrix lutea: a comparative study using light microscopy and transmission electron microscopy / Estructura histomorfológica de la Bursa cloacalis (Bursa de Fabricius) en jóvenes Leiothrix lutea: un estudio comparativo utilizando microscopía óptica y microscopía electrónica de transmisión

SUMMARY: This study aimed to investigate the microstructure and ultrastructure of the Bursa cloacalis (Bursa of Fabricius) (BC) in young Leiothrix lutea at various days of age (a few days after hatching) using light microscopy and transmission electron microscopy. The bird BC was sampled at 1, 5, 7, and 9 days of age. Immediately after dissection, the structure and integrity of the BC (not degenerative) were retained and the specific temporal features could be visualized precisely. After hematoxylin-eosin staining and uranyl acetate/lead citrate staining, the microstructure and ultrastructure of the BC, respectively, could be observed clearly. The microscopic observations revealed the following: in addition to change in the size of BC or lymphoid follicles, many cavities were found in the BC; the distribution of the lymphoid follicles in Leiothrix lutea was different from that in other birds; and the segregating line between the bursal cortex and medulla became increasingly clear as the age increased. In conclusion, the structural data obtained in this study provides a better understanding of the specific immunological function of the BC in Leiothrix lutea.

RESUMEN: Este estudio tuvo como objetivo investigar la microestructura y ultraestructura de la Bursa cloacalis (BC) en Leiothrix lutea joven unos días después de la eclosión, utilizando microscopía óptica y microscopía electrónica de transmisión. La BC se muestreó a los 1, 5, 7 y 9 días de edad del Leiothrix lutea. Inmediatamente después de la disección, se observó la estructura y la integridad de la CB (no degenerativa) y se pudo visualizar con precisión las características temporales específicas. Después de la tinción con hematoxilina-eosina y con acetato de uranilo /citrato de plomo, pudimos observar claramente la microestructura y ultraestructura de la BC. Las observaciones microscópicas revelaron el cambio en el tamaño de la CB o de los folículos linfoides y además, se encontraron numerosas cavidades en la CB; la distribución de los folículos linfoides en Leiothrix lutea era diferente a la de otras aves; y la línea de segregación entre la corteza bursal y la médula se hizo cada vez más clara a medida que aumentaba la edad. En conclusión, los datos estructurales obtenidos en este estudio proporcionan una mejor comprensión de la función inmunológica específica de la Bursa cloacalis en Leiothrix lutea.

Clonality test by PCR - PARR in real time of canine lymphomas

Lymphoma is a neoplasm of hematopoietic origin that affects canines. The proper establishment of prognosis and rapid institution of treatment are essential for a better quality of life, and immunophenotyping is one of the tools used for this purpose. The objective of this study was to perform a clonality test for immunophenotypic characterization of canine lymphomas using the polymerase chain reaction (PCR) for antigen receptor rearrangements (PARR) technique in real-time from samples fixed in formalin and embedded in paraffin. The 23 analyzed samples were fixed in formalin and embedded in paraffin canine lymphoma from the collection Laboratory of Histopathology of the Animal Pathology Area of the Departament of Veterinary Medicine - Federal Rural University of Pernambuco (UFRPE). Samples were processed, their DNA was extracted, quantified, diluted, and standardized at a concentration of 50 ng/µL. After extraction, all samples were subjected to conventional PCR for endogenous control (detection of the IgM target region), in which the extracted DNA was amplified in a final volume of 25 µL. The 128 bp amplified product was detected by 1.5% agarose gel electrophoresis. Of the 23 samples analyzed for the detection of the conserved region referring to the endogenous gene, 91.30% (21/23) amplified the conserved region Cµ by conventional PCR, and two samples 8.70% (2/23) were negative. Endogenous control positive samples were subjected to real-time PCR-PARR for detection of IgH Major and IgH Minor for B lymphocytes (LB), and TCRy for lymphocytes T (LT) target regions. All reactions were performed in duplicate to reduce the risk of false-positive or false-negative results due to technical errors. Samples previously confirmed by immunohistochemistry were used as positive controls for T cell and B cell lymphoma, and MilliQ water was used as a negative reaction control. After amplification, the melting curve gradually increased the temperature by 1o C/5 s to 95o C during continuous fluorescence monitoring. Of the 21 samples analyzed, 100.00% (21/21) demonstrated clonal amplification. Of these, 57.15% (12/21) were positive for phenotype B, and 42.85% (9/21) were positive for phenotype T. Due to the importance of researching and confirming samples from files fixed and embedded in paraffin samples in laboratories, PCR-PARR is a good tool for this purpose. In the present study, real-time PCR analysis demonstrated greater sensitivity in the characterization of the immunophenotype of lymphomas from old samples fixed in formalin and embedded in paraffin. The temperature of melting curve analysis may vary depending on the amount of DNA and its quality. In the present study, it was found that the average melting temperature in the samples varied between ± 3o C when compared to that in the control sample for LB and LT, 83.5o C and 80o C, respectively: in the literature, there is a relative difference in this temperature, which may vary up to 4o C. Real-time PCR-PARR was satisfactory in the characterization of the immunophenotype of canine lymphomas from formalin-fixed and paraffin-embedded samples; therefore, its use is recommended for both retrospective studies. The use of PCR-PARR associated with histopathological and/or cytopathological examination in cases of canine lymphomas strongly helps pathologists, provide a safe establishment of the immunophenotype, minimize errors, and optimize the diagnosis, thus directly contributing to the establishment of the prognosis.(AU)

Amígdalas y adenoides: defensa ante agentes patógenos y su implicancia estomatológica / Tonsils and adenoids: defend to pathogenic agents and their stomatological involvement

La función de las amígdalas siempre ha sido discutida, desde afirmar que no tenían funcionalidad, hasta la actualidad que se plantea un papel inmunológico, con actividad linfocitaria de defensa, debido a la localización de linfocitos en el tejido de las amígdalas. Este artículo de actualización pretende describir desde la embriología, histología, fisiología, patología y estomatología, el rol que desempeñan las mismas en su papel inmunológico ante la acción de agentes patógenos. Se destaca la acción conjunta de las amígdalas palatinas, amígdalas faríngeas o adenoides, amígdalas peritubarias, amígdalas linguales y todo el resto de tejido linfático que conforman el anillo linfático faríngeo o anillo de Waldeyer, ya que cumplen un rol determinante en la defensa del organismo (AU)

The function of the tonsils has always been debated, from stating that they had no functionality, to the present day that an immunological role is proposed, with lymphocyte defense activity, due to the location of lymphocytes in the tissue of the tonsils. This update article aims to describe from embryology, histology, physiology, pathology and stomatology, the role they play in their immunological role against the action of pathogens. The joint action of the palatine tonsils, pharyngeal or adenoid tonsils, peritubal tonsils, lingual tonsils and all the rest of the lymphatic tissue that make up the pharyngeal lymphatic ring or Waldeyer's ring is highlighted, since they play a decisive role in the defense of the organism (AU)

Revision of Laboratory Testing Guidelines for Initial Diagnosis of Hematologic Neoplasms

Metástasis solitaria intraparenquimatosa del bazo estudios clínicos patológicos e inmunohistoquímico / Intraparenchymal solitary spleen metastasis pathological and immunohistochemical clinical studies

Las metástasis esplénicas intra-parenquimatosas de tumores sólidos son tumores muy raros, y si se trata de nódulos únicos, son excepcionales. Presentamos dos casos de metástasis intra-parenquimatosas. El primer caso se trató de una mujer de 66 años con antecedentes de un adenocarcinoma endometrial. El segundo caso se trató de un paciente de sexo masculino con antecedente de un adenocarcinoma prostático, quien se presentó con un adenocarcinoma mucinoso en el bazo, como segunda neoplasia. También realizamos una revisión de los registros de biopsias de 14 años, del servicio de anatomía patológica del instituto(AU)

The spleen parenchymal metastasis of the solid tumors, are very rare, but the isolated metastasis to the spleen are considered exceptional. We herein present two cases of the isolated splenic metastases. The first case was a female a 66 year old female with a diagnosis endometrial adenocarcinoma. The second cases was a 71 years old male, with a history of a prostatic adenocarcinoma, and a spleen metastasis of a ucinous adenocarcinoma, as a second malignancy. We also made a review of 14 years, biopsies records from the pathology service, of the institute(AU)

Clinical Efficacy of Radiotherapy in Helicobacter pylori Negative or Unresponsive to Eradication Therapy Primary Gastric Mucosa-Associated Lymphoid Tissue Lymphoma / 대한소화기학회지

BACKGROUND/AIMS: The eradication of Helicobacter pylori (H. pylori) is an effective treatment in gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated with H. pylori infection. However, the treatment strategy in gastric MALT lymphoma patients who are H. pylori-negative or unresponsive to H. pylori eradication therapy remains controversial. In this study, we investigated the clinical efficacy of treatments other than H. pylori eradication therapy in these groups of patients. METHODS: This was a retrospective single-center study based on the medical records of patients diagnosed with gastric MALT lymphoma at Yeungnam University Medical Center between January 2005 and December 2016. Patients were treated with H. pylori eradication therapy, chemotherapy, or radiotherapy according to their H. pylori infection status and stage of gastric MALT lymphoma. RESULTS: Of the 68 eligible patients, 50 were enrolled in the study. Of the 42 patients with H. pylori-positive gastric MALT lymphoma, 36 (81.7%) were treated with H. pylori eradication therapy as primary treatment and 25 (69.4%) achieved a complete response (CR). Patients without a CR after H. pylori eradication therapy (n=11, 30.6%) received radiotherapy as a secondary treatment. Two patients with H. pylori-positive gastric MALT lymphoma and eight with H. pylori-negative gastric MALT lymphoma received radiotherapy as the primary treatment. CR was achieved in all 21 patients treated with radiotherapy as primary or secondary treatment. The 5-year progression-free survival rate after radiotherapy was 92.9%. CONCLUSIONS: Radiotherapy may be a worthwhile treatment option in patients with H. pylori-negative MALT lymphoma or H. pylori-positive MALT lymphoma that is not responsive to H. pylori eradication therapy.

Mucosa-associated lymphoid tissue lymphoma on right lower eyelid previously diagnosed as lymphoid hyperplasia

Mucosa-associated lymphoid tissue (MALT) lymphoma is an uncommon form of non-Hodgkin lymphoma involving the mucosa-associated lymphoid tissue. Most commonly affected organ is the stomach. But, it could affect almost every organ in the whole body. If they have suspicious lesion, excision biopsy could be made. For staging, blood test including blood smear, abdomen and chest tomography or scan will be checked. Also, bone marrow test can be done if it is needed. The patient had visited the clinic for palpable mass on right lower eyelid. With excisional biopsy, it was diagnosed as lymphoid hyperplasia on pathologic test. But 2 years later, the patient came with recurrent symptom for our department with worry. At that time, we recommended excisional soft tissue biopsy under general anesthesia. Unfortunately, it was revealed MALT lymphoma on pathologic finding. It turned out to be stage 3 in Ann Arbor staging system without B symptoms. Hematologic consultation was made and she was treated with adjuvant chemotherapy for eight cycles to complete remission. We report a case of MALT lymphoma on subcutaneous tissue at right lower eyelid previously diagnosed as lymphoid hyperplasia.

Lower Risk of Gastric Atrophy and Intestinal Metaplasia in Patients with MALT Lymphoma despite Helicobacter pylori Infection

BACKGROUND/AIMS: Atrophic gastritis and intestinal metaplasia are sequential consequences of chronic Helicobacter pylori (H. pylori) infection. These conditions are well known to increase the risk of gastric adenocarcinoma development. Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is also a malignant consequence of H. pylori infection, but the relationship between gastric MALT lymphoma and atrophic gastritis-intestinal metaplasia has not been a focus of interest. We investigated the clinical characteristics of atrophic gastritis and intestinal metaplasia in patients with gastric MALT lymphoma. MATERIALS AND METHODS: A study was conducted by reviewing the electronic medical records of patients diagnosed as having gastric MALT lymphoma at an academic institute, the Yeouido St. Mary's Hospital, Seoul, Korea, between January 2001 and December 2018. RESULTS: Fifty-eight subjects were enrolled consecutively during the study period and analyzed retrospectively. The patients' mean age was 56.9 years old. The male-to-female ratio was 1.15 (31/27). On histological examination, background atrophic gastritis and intestinal metaplasia were detected in 26.8% (15/58) of cases. Serum pepsinogen I, II and gastrin levels, as serological markers of atrophy, were evaluated in 28 subjects. Three (5.2%) of the 28 cases were compatible with serological atrophic gastritis (pepsinogen I/II ratio of <3 and pepsinogen I level of <70 ng/mL). CONCLUSIONS: In patients with gastric MALT lymphoma, the prevalence of background mucosal atrophy or intestinal metaplasia was 26.8% on histological examination and 5.2% on serological analyses. These rates are lower than those in patients with gastric adenocarcinoma. This result suggests a different carcinogenic pathway of gastric MALT lymphoma from that of adenocarcinoma.

Nodular Gastritis as a Precursor Lesion of Atrophic and Metaplastic Gastritis / 대한소화기병학회잡지

BACKGROUND/AIMS: Chronic atrophic gastritis (CAG) and metaplastic gastritis (MG) are precancerous conditions of Helicobacter pylori (H. pylori)-related gastric cancer. This study aimed to identify the characteristics of nodular gastritis (NG) showing CAG or MG after nodule regression. METHODS: H. pylori-infected patients with NG were included after upper gastrointestinal endoscopy. Patients were excluded if their latest endoscopy had been performed ≤36 months after the initial diagnosis of NG. Small-granular-type NG was defined as the condition with 1–2 mm regular subepithelial nodules. Large-nodular-type NG was defined as those with 3–4 mm, irregular subepithelial nodules. The endoscopic findings after nodule regression were recorded. RESULTS: Among the 97 H. pylori-infected patients with NG, 61 showed nodule regression after a mean follow-up of 73.0±22.0 months. After nodule regression, 16 patients showed a salt-and-pepper appearance and/or transparent submucosal vessels, indicating CAG. Twenty-nine patients showed diffuse irregular elevations and/or whitish plaques, indicating MG. Sixteen patients with other endoscopic findings (14 normal, one erosive gastritis, and one chronic superficial gastritis) showed a higher proportion of H. pylori eradication (12/16, 75.0%) than those in the CAG group (5/16, 31.3%) and MG group (6/29, 20.7%; p=0.001). Patients with small-granular-type NG tended to progress toward CAG (14/27, 51.9%), whereas those with large-nodular-type NG tended to progress toward MG (25/34, 73.5%; p<0.001). CONCLUSIONS: In patients with a persistent H. pylori infection, NG tended to progress to CAG or MG when the nodules regressed. Small-granular-type NG tended to progress to CAG, whereas large-nodular-type NG tended to progress to MG.

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.

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.

Endoscopic features aiding the diagnosis of gastric mucosa-associated lymphoid tissue lymphoma / 영남의대학술지

The incidence of gastric mucosa-associated lymphoid tissue (MALT) lymphoma is increasing worldwide, but the diagnosis is difficult. Most patients are asymptomatic or complain of nonspecific gastrointestinal symptoms. As the endoscopic features of gastric MALT lymphoma are variable and nonspecific, the possibility of this condition may be overlooked during esophagogastroduodenoscopy, and it remain undiagnosed. Therefore, this condition needs to be considered when an abnormal mucosa is observed during this procedure. Biopsy performed during endoscopy is the primary diagnostic test, but false negative results are possible; large numbers of samples should be collected from both normal and abnormal mucosae. Endoscopic ultrasonography is useful to assess the depth of invasion and to predict the treatment response. After treatment, follow-up tests are required every 3 months until complete remission is achieved, and annually thereafter. Early diagnosis of gastric MALT lymphoma is difficult, and its diagnosis and follow-up require wide experience and competent endoscopic technique.

Nodular Gastritis as a Precursor Lesion of Atrophic and Metaplastic Gastritis / 대한소화기학회지

BACKGROUND/AIMS: Chronic atrophic gastritis (CAG) and metaplastic gastritis (MG) are precancerous conditions of Helicobacter pylori (H. pylori)-related gastric cancer. This study aimed to identify the characteristics of nodular gastritis (NG) showing CAG or MG after nodule regression.METHODS: H. pylori-infected patients with NG were included after upper gastrointestinal endoscopy. Patients were excluded if their latest endoscopy had been performed ≤36 months after the initial diagnosis of NG. Small-granular-type NG was defined as the condition with 1–2 mm regular subepithelial nodules. Large-nodular-type NG was defined as those with 3–4 mm, irregular subepithelial nodules. The endoscopic findings after nodule regression were recorded.RESULTS: Among the 97 H. pylori-infected patients with NG, 61 showed nodule regression after a mean follow-up of 73.0±22.0 months. After nodule regression, 16 patients showed a salt-and-pepper appearance and/or transparent submucosal vessels, indicating CAG. Twenty-nine patients showed diffuse irregular elevations and/or whitish plaques, indicating MG. Sixteen patients with other endoscopic findings (14 normal, one erosive gastritis, and one chronic superficial gastritis) showed a higher proportion of H. pylori eradication (12/16, 75.0%) than those in the CAG group (5/16, 31.3%) and MG group (6/29, 20.7%; p=0.001). Patients with small-granular-type NG tended to progress toward CAG (14/27, 51.9%), whereas those with large-nodular-type NG tended to progress toward MG (25/34, 73.5%; p<0.001).CONCLUSIONS: In patients with a persistent H. pylori infection, NG tended to progress to CAG or MG when the nodules regressed. Small-granular-type NG tended to progress to CAG, whereas large-nodular-type NG tended to progress to MG.

Aspectos patológicos e imuno-histoquímicos da polisserosite em búfalos (Bubalus bubalis) / Histopathological and immunohistochemical study of the polyserositis in buffalo (Bubalus bubalis)

Polisserosites são alterações inflamatórias das serosas viscerais e parietais das cavidades corpóreas. Um tipo especial destas alterações foi identificado em bubalinos abatidos para consumo nos anos 80, sendo associada a infecção por Chlamydia psittaci. Apesar da importância da bubalinocultura no Pará, do caráter zoonótico da C. psittaci e da possibilidade de envolvimento de outros agentes na afecção, são raros trabalhos sobre a enfermidade desde os estudos pioneiros. No presente trabalho casos identificados como polisserosite pelo serviço de inspeção sanitária em búfalos abatidos para consumo foram coletados com objetivo de determinar a frequência e origem dos mesmos e de caracterizar as lesões e pesquisar antígenos de Chlamydia spp. nas mesmas. De um total de 2.887 bubalinos abatidos no período de estudo foram identificados 48 (1,66%) casos. O município de Santa Cruz do Arari na Ilha de Marajó apresentou a maior porcentagem de casos em relação ao número de animais abatidos (6,49%, 5/77). Na macroscopia as lesões se caracterizaram por áreas opacas, branco-amareladas de espessamento das serosas, por vezes com franjas fibrosas na superfície. Os resultados permitem concluir que os casos classificados como polisserosite pelos veterinários da inspeção sanitária nos búfalos abatidos para consumo correspondem na histopatologia a um tipo incomum de polisserosite, caracterizada por infiltrado linfocitário, com ocasionais formações de folículos linfoides terciários ou ectópicos. Foi observado ainda nestas lesões um predomínio de linfócitos T (CD3 positivos) no infiltrado, sendo os linfócitos B (CD79 positivos) presentes em maior número no interior das estruturas foliculares. Não foram demonstrados antígenos de clamídias nas lesões do presente estudo, o que demonstra a necessidade de estudos adicionais relativos a(s) etiologia(s) das lesões.(AU)

Polyserositis are inflammatory changes of the visceral and parietal serous of body cavities. A special type of polyserositis was identified in buffaloes in the 80s, being associated with infection by Chlamydia psittaci. Since these pioneering studies, there are no additional works about the condition. Considering the importance of buffalo in Pará, the zoonotic character of C. psittaci and the possibility of involvement of other agents in polyserositis in buffaloes the present study is proposed. We collected cases identified as polyserositis by sanitary inspection service in buffalo slaughtered for consumption in Belem for a complementary characterization of inflammatory cell and the research of Chlamydia spp antigens in lesions. Of 2.887 buffaloes slaughtered in a period of six months, there were 48 (1.66%) cases of polyserositis and 39 analyzed. Santa Cruz do Arari in Marajó Island was the city with the highest frequency of cases, whereas 6.49% of buffaloes had lesions. However, 50% of the present study cases came from Soure municipality in Marajó Island, which provided about 49% of buffaloes slaughtered in the period. In the macroscopy, there were opaque areas with white-yellow thickening of the serous, sometimes with fibrous fringes on the surface. Histopathology showed connective tissue projections partially lined by cuboid or flattened mesothelial cells. Often in projections there were mononuclear infiltrate of variable intensity, consisting mainly of lymphoid cells, with occasional ectopic or tertiary lymphoid follicles.(AU)

Major Changes to the 2017 Revision of the World Health Organization Classification of Myeloproliferative Neoplasms / 대한내과학회지

The World Health Organization (WHO) Classification of Tumors of Haematopoietic and Lymphoid Tissues was recently published in a revised fourth edition. The categories of myeloproliferative neoplasms (MPNs) have not significantly changed since the 2008 fourth edition of the classification; however, newly discovered mutations including CALR and CSF3R and improved characterizations and standardizations of morphological features of some entities, particularly BCR-ABL1-negative MPNs, have impacted the diagnostic criteria of disease entities, increasing the reliability and reproducibility of diagnoses. The 2017 revised edition attempts to incorporate new clinical, prognostic, morphologic, and genetic data that have emerged since the last edition. This article reviews the major changes in the classification and their rationale for MPN classification within the revised 2017 WHO system.

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.

A Case of Orbital Lymph Node Misdiagnosed as a Dermoid

PURPOSE: We report our experience with a case of orbital lymph node which has not been previously reported in the Republic of Korea. CASE SUMMARY: A 24-year-old female patient with no underlying disease visited our hospital with a 2-month history of a mass at the lateral side of the left upper eyelid. On physical examination, a round, well-defined subcutaneous mass was palpable, and pain, swelling or a skin change were not reported or seen. The appearance, location, and imaging findings of the mass were suspected to be dermoid, and excisional biopsy was performed for accurate diagnosis and treatment. The 1.0 × 0.7 cm-sized, round-shaped and brown-colored mass was excised. The mass was well-defined but in the posterior part; it was attached to the periosteum. The mass was finally diagnosed as a lymph node with lymphadenitis by histopathological examination. No recurrence or new lesion was observed after surgery, and the patient did not complain of abnormal symptoms. CONCLUSIONS: A Complete orbital lymphoid tissue has not yet been found in the orbit, and lymph nodes are known to be absent. However, as in this case, when an orbital mass is observed, the possibility of lymph node should be considered and histopathological examination should be performed to confirm it.

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).