Synchronous Presentation of Two Extranodal Lymphomas: Follicular Lymphoma and Extranodal Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue (MALToma)

Synchronous malignancies are rare conditions in oncology practices, generally seen as solid tumors with hematological neoplasms. However, occurrence of two different hematological malignancies in the same patient is extremely rare. Two primary malignancies should be considered especially in patients with extraordinary presentations and treatment resistance

Survival outcomes of hypomethylating agents maintenance therapy in new diagnosed AML patients: Real experience data

OBJECTIVE: Acute myeloid leukemia (AML) is a hematological malignancy that frequently affects elderly population. With introducing the hypomethylating agents (HMAs) in elderly AML treatment, survival rates and quality of life have improved. However, long-term management in elderly and frail patients is still a challenge. In the present study, we aimed to determine whether HMA maintenance therapy is required until disease progression in frail and elderly AML patients by examining with a real-life data.METHODS: In a multicenter study, we analyzed non-promyelocytic elderly AML patients who were treated with first-line azacitidine or decitabine monotherapy in two different groups, retrospectively. While patients were treated with HMA until progression in the maintenance group, 6+3 cycles of azacitidine or decitabine were administered as a standard care of elderly AML patients in the non-maintenance group. Survival outcomes were compared between the groups.RESULTS: HMA therapy was maintained until progression in 20 patients, and HMA therapy was terminated after 6+3 cycles in 21 patients. Patients received a median of 6 (1-14) HMA cycles during follow-up time. The median 7.5 months of overall survival were observed (2-17 months) in maintenance and 3 months (1-13 months) in non-maintenance groups (p=0.001).CONCLUSION: Despite long-term exposure to HMA may appear as a risk factor for complications and toxicities in elderly and frail AML patients, the maintenance of therapy until disease progression provides a significant survival advantage. Therefore, we suggest that HMA therapy should continue until disease progression regardless the sort of HMA

A Stranger in the Epididymis: Ectopic Spleen

Splenogonadal fusion, which can be clinically confused with testicular neoplasms as they present with testicular swelling, is a rare abnormality in young adults. Intraoperative frozen section analysis can be helpful in diagnosing such cases and preventing unnecessary orchiectomies. We report a case in which frozen section analysis was done and testis-sparing surgery could be performed

Gender-related Differences in Surgically Treated Patients with Renal Cell Carcinoma

Objective:The aim of the study was to investigate gender-specific differences in the Turkish patients with renal cell carcinoma (RCC) undergoing radical or nephron-sparing nephrectomy and compare the results with those in other regions.Materials and Methods:Data of 76 patients, who were clinically diagnosed with RCC and underwent radical or nephron-sparing nephrectomy from January 2011 to August 2017, were retrospectively evaluated. Age and gender of the patients and the size, histological type, grade and pathological stage of the tumors were recorded. A chi-square test was used for comparing categorical variables, whereas the Student’s t-test was used for the same purpose in the continuous variables.Results:Of the 67 patients, 39 (58.2%) were male and 28 (41.8%) were female; male-to-female ratio was 3:2. The mean age of the male and female patients was 63.4±11.7 years and 59.3±14.3 years, respectively and the mean tumor size was 5.7 and 5.3 cm, respectively. There were differences in mean age, tumor size and Fuhrman grade, however, none of them reached the level of statistical significance. Twenty four of the male and 25 of the female patients had low-stage, 15 of the male and 3 of the female patients had high-stage disease. Thus, the male patients had higher stage disease than the female patients and the difference was statistically significant (p=0.011).Conclusion:Turkish women with RCC had significantly lower stage disease than Turkish men, although grade and size of the tumor did not present a statistically significant difference. The results were similar with other European studies

The Characteristic Clinical and Pathological Features of Incidental and Non-Incidental Papillary Microcarcinoma of the Thyroid

The incidence of papillary microcarcinoma (PMC) keeps raising due to fine needle aspiration (FNA) biopsies and the pathologic evaluation of thyroid specimens of cases operated for benign thyroid disorders. The files of 78 patients who underwent surgery between January 2006 and May 2013 in our department and were diagnosed with PMC were analyzed, retrospectively.Cases were grouped as incidental and non-incidental depending on the preoperative diagnosis. The diameter of the nodules in the preoperativeultrasonographic examination, pathological parameters such as tumor size, bilaterality, multifocality,and capsular invasion were found significantly high in non-incidental group(P<0.05). No significant difference was detected between the two groups’ age, sex, and vascular invasion.Preoperative radiologic examination by experienced radiologists and FNA performed for suspicious nodules may increase the rate of early and correct diagnosis. In our opinion, determining the most useful criteria for malignancy will help to detect incidental PMC in cases followed up for multinodular goiter

Kaleidoscopic Views in the Bone Marrow: Oxalate Crystals in a Patient Presenting with Bicytopenia

Pancytopenia associated with BM infiltration of different deposits is a rare condition mostly associated with amyloidosis or the accumulation of iron. One of the rarest deposits in the BM is oxalate crystals due to hyperoxaluria [1,2,3]. Primary hyperoxaluria, a genetic disorder due to mutation in the alanine glyoxylate aminotransferase gene, located on chromosome 2q37.3 and resulting in the conversion of glyoxylate to oxalate, is characterized by increased production of oxalic acid because of the specific liver enzyme deficiency and generally presents with renal stones, renal or liver failure, and oxalosis [4]. Calcium oxalate may even be deposited into various tissues such as those of the retina, peripheral nerves, arterial media, and heart [4,5]. The medical history of nephrolithiasis at early ages, characteristic appearance of birefringent crystals forming rosettes in the BM, and the envelope-like forms in the BM aspirates seen in our case supported the diagnosis of primary hyperoxaluria, which is best confirmed by genetic studies and treated with liver transplantation because of the location of the abnormal enzymes in the hepatocytes

Gaucher disease in an adult: A rare cause of hepatosplenomegaly in adults

WOS: 000433094100012PubMed ID: 2960743

Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease

Rosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment

Different clinical courses with the same findings: two cases of paroxysmal nocturnal hemoglobinuria presenting with thrombocytopenia

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal stem cell disease that manifests with chronic intravascular hemolysis, thrombosis, and bone marrow failure. Various degrees of cytopenias accompany the disease. Although laboratory and clinical findings are similar, the disease may show different courses and require different treatments. Herein, we report two different courses of PNH with similar clinical and laboratory findings