Systemic Amyloidosis in a Patient With Familial Mediterranean Fever and Hodgkin Lymphoma: A Case Report.

Systemic amyloidosis is a clinical manifestation of the accumulation of amyloid fibrils in tissues because of persistent acute phase elevation and chronic inflammation. Its most common causes are inflammatory diseases and malignancies. Here, we present a 12-year-old girl diagnosed with systemic amyloidosis and Hodgkin lymphoma (HL) who was also previously diagnosed with familial Mediterranean fever (FMF). Despite colchicine treatment for FMF, the patient had a persistent elevation of acute phase reactants and AA-type amyloid deposits were observed in a kidney biopsy. Anakinra, an interleukin-1 antagonist, was added to the treatment. Shortly after the diagnosis of amyloidosis, mediastinal lymphadenopathy was recognized, and she was also diagnosed with HL. A chemotherapy protocol of doxorubicin, bleomycin, vinblastine, and dacarbazine was initiated. After 6 cycles of the chemotherapy and 8 months of the anakinra treatment, no recurrence or residual malignancy was observed and proteinuria was decreased. To the authors' knowledge, this is the first reported case of systemic amyloidosis in the literature associated with both FMF and HL.

Comparison of the effect of isotretionin and alitretionin on endometriotic implants and serum vascular endothelial growth factor level: an experimental study.

OBJECTIVE: To compare the effects of alitretionin and isotretionin on endometrial peritoneal implants and serum vascular endothelial growth factor (VEGF) levels. STUDY DESIGN: Forty-eight female Sprague Dawley rats were used. Initially surgical rat endometriosis model was done. The endometrial implant volume was measured and rats were randomly divided into four groups. Group 1: Control group (rats did not get any drug but having endometriotic implants), group 2: rats receiving po isotretionin 10 mg/kg per day for 10 d, group 3: rats receiving po isotretionin 20 mg/kg per day for 10 d and group 4: rats receiving po alitretionin 80 mg/kg per day for 10 d. After 1-week medication, rats were sacrificed and size, histopathology of endometriotic implant and levels of VEGF were evaluated. RESULTS: Volumes of peritoneal endometrial implants were significantly decreased in Group 2 and Group 3 compared with initial values. However, there were no significant changes in histopathological scores and serum VEGF levels in all groups. CONCLUSIONS: This study finding may suggest the possible medical treatment modality of isotretionin on endometriosis. However, alitretionin (potent retinoid) does not have potent regressive effect on endometriotic implants as in isotretionin.

Nuclear morphometric findings in undetermined cytology: A possible clue for prediction of BRAF mutation in papillary thyroid carcinomas.

PURPOSE: To investigate the possible relationship between the nuclear morphometric characteristics (nuclear perimeter, roundness, nuclear area, and nuclear shape) and BRAF mutation status in papillary thyroid carcinoma cases with a prior diagnosis of undetermined cytology. MATERIALS AND METHODS: Total thyroidectomy specimens obtained from 48 patients with papillary thyroid carcinoma with a prior diagnosis of undetermined cytology were included. Morphometric analysis under light microscopy included measurements of the perimeter, shape factor, nuclear area, and roundness of thyrocyte nuclei from the cytological smear preparations. Mutational analysis, including immunohistochemistry and polymerase chain reaction, was performed in formalin fixed paraffin embedded tissue blocks. RESULTS: BRAF V600E mutation was detected in 7 of 48 cases (14.5%). The nuclear perimeter and nuclear area in mutated cases were significantly higher than the wild type (p = 0.005). Shape factor (p = 0.681) and roundness (p = 0.752) values did not significantly differ between the wild-type and mutant groups. No significant relationship was evident between BRAF expression and BRAF point mutation. CONCLUSION: In cases with positivity for BRAF mutation, the nuclear perimeter and nuclear area were significantly increased. These findings suggest that morphometric variables are predictive markers for papillary thyroid carcinoma cases with positivity for BRAF mutation. However, further trials on larger series are warranted to understand the significance and predictive value of nuclear morphometric analysis in these circumstances.

[A rare case: Kikuchi-Fujimoto disease]. / Nadir görülen bir olgu: Kikuchi-Fujimoto hastaligi.

Kikuchi-Fujimoto's disease (CFD) is a disease that usually exhibits self-limited benign lymph node involvement. Etiology of illness is not fully understood.Differential diagnosis should be performed well because lymph node involvement can be confused with lymphoma type diseases. A 35-year-old male patient with weakness, joint aches, fever, loss of appetite, weight loss, night sweating symptoms presented with the literature.

Hormone-secreting large adrenal ganglioneuroma in an adult patient: a case report and review of literature.

BACKGROUND. Ganglioneuromas (GNs) are neural crest cell-derived tumors and rarely occur in the adrenal gland. They are usually asymptomatic and hormonally silent. The majority of cases are detected incidentally during work-up for unrelated conditions. Hormone-secreting pure adrenal GNs in adults are extremely rare. To date, only four cases have been reported in the English literature. CASE REPORT. We describe an adult case of endocrinologically active adrenal GN incidentally diagnosed in a 64-year-old male patient with history of uncontrolled hypertension. On physical examination, he had a blood pressure (BP) of 160/100 mmHg. Abdominal computed tomography and magnetic resonance imaging showed a large solid tumor (8.5 × 7.5 × 7 cm) in the right adrenal gland. Urinary levels of norepinephrine, normetanephrine, vanillylmandelic acid and dopamin were elevated, although urinary level of epinephrine was suppressed. Right adrenalectomy was performed for treatment purposes. The histological diagnosis of the resected tumor was adrenal GN. CONCLUSIONS. Hormone-secreting pure adrenal GN occurs very rarely in adults and preoperative diagnosis is difficult. Adrenal GN may present with hormonal activity such as increased secretion of catecholamines and their metabolites. There are no specific diagnostic signs and symptoms discriminating GN and pheochromocytoma. Therefore, histopathological examination need for a definitive diagnosis of adrenal GN. The prognosis after completed surgical resection without further therapy seems to be excellent. To our knowledge, the present case is the second report that describes hormone-secreting pure adrenal GN in an adult from Turkey in the English literature. We discuss this case and review the literature on this unusual entity.

Spitz nevus arising upon a congenital glomuvenous malformation.

There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.

PD-L1 expression in invasive solid papillary breast carcinomas.

Background: Invasive solid papillary carcinomas (ISPC) are rare malignant neoplasms in the classification of WHO 2019 breast tumors. Aims: We aimed to investigate the correlations between programmed cell death ligand-1 (PD-L1) expression status of tumor and immune cells and clinicopathological parameters by molecular classification of this rare morphological subtype. This study will contribute to the literature about the PD-L1 expression state of ISPCs for the first time. Material and Methods: The study included 19 invasive solid papillary carcinoma cases diagnosed between 2009 and 2019 in Pathology Department. Molecular subtyping was performed in 19 cases by immunohistochemical studies (ER/PR, Her-2/neu, Ki-67), and PD-L1 expression was evaluated in neoplastic and immune cells. Results: PD-L1 expression was detected in 4 (21%) cases, 3 (75%) of them were in luminal B and 1 (25%) were in the luminal A group. The correlation between molecular subtypes and PD-L1 expression was statistically significant (P = 0.016). Patients with PD-L1 expression had a higher Ki-67 index than patients without PD-L1 expression (P = 0.037). In addition, there was a statistically significant correlation between PD-L1 expressions of intratumoral lymphocytes and PD-L1 expressions of neoplastic cells (P = 0.004). Conclusions: While predicting the group that will benefit more from immunotherapy in solid papillary carcinoma cases, not only PD-L1 expression of tumor cells but also PD-L1 expression in tumor infiltrating lymphocyte (TIL) can help. In addition, PD-L1 staining rates of tumor cells as well as clinicopathological parameters (molecular subtype, high Ki-67 index, presence of TIL) can be predictive about immunotherapy.

Could Amifostine Prevent Experimental Radiotherapy-Induced Acute Pericarditis?

BACKGROUND: Amifostine is a powerful antioxidant that is one of the documented three chemo-radio prototectants recommended for clinical use. There is no data exploring amifostine in prevention of acute pericardial damage. We aimed to investigate whether amifostine has protective effect against acute pericardial injury due to radiotherapy in an experimental rat model. METHODS: Twenty-four rats were divided into four groups: control group, radiotherapy-only group, amifostine-only group, radiotherapy+amifostine group. In groups receiving radiotherapy, hearts were irradiated with a Co 60 teletherapy device at a distance of 80 cm and 20 Gy at a depth of 2 cm. Thirty minutes before interventions, 200 mg/kg amifostine or same volume 0.9% NaCl were administered intraperitoneally. Subjects were sacrificed 24 hours after the procedure. Pericardial histopathological changes were investigated by light microscopy. RESULTS: There was focal inflammation of >= 50% in all rats exposed-to-radiotherapy. All groups receiving radiotherapy revealed a significant increase in pericardial inflammation compared to the groups that did not receive irradiation (p<0.05). There was no difference between the radiotherapy-only group and amifostine+radiotherapy group for pericardial inflammatory response (p>0.05). CONCLUSION: Acute pericarditis was detected in all rats receiving radiotherapy. There was no positive effect of amifostine administration before radiotherapy on acute pericardial inflammation.

MDCT with multiplanar reconstruction in the preoperative local staging of rectal tumor.

OBJECTIVE: To evaluate the accuracy of MDCT with multiplanar reconstruction in the preoperative local staging of rectal tumor. MATERIALS AND METHODS: Thirty-seven patients with rectal tumor underwent preoperative MDCT. Two radiologists evaluated the depth of tumor invasion (T staging), regional lymph node involvement (N staging) and mesorectal fascia involvement on axial, sagittal, and coronal multiplanar reconstruction images in consensus. MDCT findings were compared with pathologic results, which served as the reference standard. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were assessed. RESULTS: Overall accuracy was 86% in T staging, 84% in N staging, 89% in International Union Against Cancer (UICC) Staging, and 94.5% in the prediction of mesorectal fascia involvement. CONCLUSION: MDCT with multiplanar reconstruction is an accurate technique in the preoperative local staging of rectal tumor.

Does propofol prevent testicular ischemia-reperfusion injury due to torsion in the long term?

OBJECTIVES: Our aim was to investigate the long-term preventive effect of propofol on testicular ischemia-reperfusion injury in a rat model. METHODS: Twenty-four adult male Sprague-Dawley rats were randomly divided into four groups (n = 6 for each group), control, sham-operated, torsion/detorsion (T/D) and T/D + propofol. Testicular ischemia was achieved by twisting the left testis 720° clockwise for 2 h. Half an hour before detorsion, 50 mg/kg propofol was given intraperitoneally to the T/D + propofol group. Ipsilateral orchiectomies to determine mean testicular weights and histopathological examination according to Johnsen's mean testicular biopsy score criteria were performed 30 days after surgical procedure in all groups. RESULTS: Mean testicular weights were 1.57 ± 0.12 g in group I, 1.59 ± 0.36 g in group II, 0.84 ± 0.20 g in group III and 0.87 ± 0.29 g in group IV. Mean testicular weights decreased significantly in the T/D groups, but no improvement in testicular weight was observed with propofol administration (p 0.9372). Similarly, the Johnsen's mean testicular biopsy scores of the T/D groups were lower than those of the control and sham-operated groups, but no positive effect was determined with the administration of propofol in the T/D groups (p 0.1797). CONCLUSIONS: Our results showed that there is no apparent long-term therapeutic potential attendant on using propofol in the treatment of testicular ischemia-reperfusion injury caused by testis torsion.

[Fatal sino-orbital aspergillosis in an immunocompetent case]. / Immün kompetan bir olguda fatal seyirli sino-orbital aspergilloz.

The paranasal sinus infections caused by Aspergillus spp. are usually presented clinically with mild symptoms, however they may lead to invasive disease and mortality especially in immunocompromised individuals. In this report a fatal case of sino-orbital aspergillosis developed in an immunocompetent patient has been presented. Seventy-four years old female patient was admitted to the hospital with the complaints of fever and progressively increasing headache that continued for 15 days. Due to the development of nausea, vomiting, loss of consciousness and stiff neck in the following days, cerebrospinal fluid (CSF) sample was obtained. Direct microscopic examination of the Gram and acidfast staining of the CSF sample revealed no microorganisms, no growth was detected in CSF culture and PCR amplification was negative for Herpes simplex virus and Mycobacterium tuberculosis. Since no response was achieved by empirical ceftriaxone, ampicillin and conventional anti-tuberculosis treatment and tachypnea, proptosis and progressive respiratory failure developed in the patient, she was transferred to the intensive care unit. The radiological examination revealed soft tissue lesion filling the sphenoid sinus, extending to the nasal cavity and suprasellar cistern, destruction of bones, dilated orbital vein, cavernous sinus thrombosis and infarction on left cerebral peduncule. Patient was operated and pus and fungus ball were aspirated from the openings of both sphenoid sinuses. Gomori methenamine silver, periodic acid-Schiff and haematoxylin-eosine staining of the operational material exhibited dichotomously branching hyphae. The patient was diagnosed as invasive sino-orbital aspergillosis based on the clinical, radiological and histopathological findings. Despite antifungal therapy and surgical debridement, the patient died. It should always be kept in mind that aspergillosis can develop in immuncompetent individuals. Delay in diagnosis and treatment may lead to fatality. Thus multidiciplinary approach is necessary for early diagnosis and successful treatment of aspergillus infections.

Coexistence of basal cell carcinomas and multiple sebaceous gland hyperplasias in a cyclosporine (ciclosporin)-treated renal transplant recipient.

A 55-year-old man presented with multiple, asymptomatic, yellowish papules on his face with a 4-year history, and two non-healing tumoral lesions on his nose with a 7-month history. He was a renal transplant recipient and had been treated with cyclosporine (ciclosporin) for 9 years. A biopsy from the asymptomatic, yellowish papule on the face showed sebaceous gland hyperplasia, and biopsies from the lesions on the nose revealed basal cell carcinomas. The lesions on the nose were excised. Sebaceous gland hyperplasia and skin cancers are among the cutaneous neoplasms observed in renal transplant recipients receiving cyclosporine. To our knowledge, this is the third reported case of the coexistence of basal cell carcinomas and multiple sebaceous gland hyperplasias in a cyclosporine-treated renal transplant recipient.

The expression of MCM-2 in invasive breast carcinoma: a stereologic approach.

OBJECTIVES: The aim of this study is to examine the expression of MCM-2 and conventional proliferation marker Ki-67 in breast carcinoma by stereologic technique and to compare it with various clinicopathologic parameters. METHODS: The expression of MCM-2 and Ki-67 on paraffin-embedded tumor tissue sections of patients with invasive breast carcinoma was analyzed immunohistochemically. Stereologic method was used for evaluation of the percentage of positively stained tumor cells. RESULTS: Significant positive correlation was found between the expression of MCM-2 and that of Ki-67 (r = 0.74, p < 0.001). MCM-2 and Ki-67 expression was significantly associated with histologic grade (p < 0.05), and negative correlation was observed between MCM-2 or Ki-67 expression and estrogen status (p < 0.05). No significant association was observed between MCM-2 or Ki-67 expression and patient age, tumor size, lymph node status, clinical stage and menopausal status. CONCLUSION: Our results suggest that MCM-2 expression is significantly associated with histologic grade of breast carcinoma and with cell proliferation capacity (Ki-67 labelling index). Additional studies are required using the stereologic method to compare and understand the utility of Ki-67 and MCM-2 expression in invasive breast carcinoma (Tab. 1, Fig. 4, Ref. 34). Full Text (Free, PDF) www.bmj.sk.

Electrophysiological, functional and histopathological assessments of high dose melatonin on regeneration after blunt sciatic nerve injury.

The aim of this study was to determine the curative effects of high-dose (100 mg/kg) melatonin on peripheral nerve injury. Forty male Wistar albino rats were randomized into four groups as sham, vehicle, melatonin, and ischemia and their right sciatic nerves were exposed. The process was terminated in the sham group. In the other groups, nerve injury was induced by clip compression. The vehicle group was intraperitoneally administered ethanol 0.1 cc (melatonin solvent), while the melatonin group was intraperitoneally administered a single dose of melatonin (100 mg/kg). Following the surgery, sciatic nerve functional index (SFI) was measured using walking track analysis on days 7, 14, and 21, and latency, amplitude, and muscle action potentials (MAP) field values were measured using electroneuromyography (ENMG) on day 21. Histopathologically, edema, axonal degeneration, myelin damage, and inflammatory response were evaluated in all groups. SFI values were noted to be statistically significantly different among the vehicle, melatonin, and ischemia groups, and the melatonin group showed a faster recovery. In the ENMG evaluations, higher amplitude and field values in the melatonin group indicated that melatonin accelerated peripheral nerve recovery. Histopathologically, although fibers with loss of myelin were identified in the melatonin group, the myelin sheath was preserved in general and the axonal structure was noted to be normal. A single injection of high-dose melatonin was found to preserve myelin sheath, prevent axonal loss, and accelerate functional recovery during the nerve regeneration in peripheral nerve injury.

Is It Rational to Perform Liver Resection for Patients with Intermediate and Advanced Stages of Hepatocellular Carcinoma?

This study aimed to investigate clinical characteristics of hepatocellular carcinoma and the outcome of our aggressive treatment policy which follows the Barcelona Clinic Liver Cancer (BCLC) guidance. In this study, we retrospectively analyzed data of 102 patients who were treated for hepatocellular carcinoma between January 2007 and October 2016. Male predominance (81.4%) and a median age of 61 years were observed. Cirrhosis was evident in 88.2 per cent of patients. Viral hepatitis (77.5%) was the most common underlying etiology. The majority of our patients (71.6%) were in BCLC B and C stages. Liver resection was performed in 53.4 per cent of patients in those stages. Transarterial chemoembolization was the leading interventional treatment. Overall survival rates at three and five years were 75 per cent and 75 per cent in BCLC 0, 69 per cent and 58 per cent in BCLC A, 50 per cent and 41 per cent in BCLC B, and 11 per cent and 11 per cent in BCLC C, respectively. The BCLC treatment algorithm should consider the role of liver resection also for intermediate stages.

Association of gastrointestinal stromal tumor and acute myeloid leukemia preceded by myelodysplastic syndrome with refractory anemia.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. GISTs are believed to be related to mutational activation of receptor tyrosine kinases, KIT, or platelet-derived growth factor receptor-alpha. The coexistence of GISTs with other neoplasms has been extensively addressed in the literature. The most common second neoplasms are colorectal cancer, prostate cancer, and neoplasms derived from lymphoid tissue. In this case report, we describe a patient affected by GIST and acute myeloid leukemia preceded by myelodysplastic syndrome with refractory anemia. The clinicopathological characteristics of the patient are discussed and the literature is reviewed.

Clear cell hidradenoma of the gluteal region: a case report.

Clear cell hidradenoma is a rare skin appendage tumor. A 41-year-old female presented with right gluteal mass. Excisional biopsy of the mass was performed. Under the epidermis, an eosinophilic-cytoplasm, uniform-appearance, oval-round-nucleus, benign tumor with cystic and solid components was detected. These results were consistent with clear cell hidradenoma. The patient had not been given postoperative adjuvant treatment and has been under follow up free from disease for 2 years.

The association of bladder myeloid sarcoma and unclassified myelodysplastic/myeloproliferative disease.

Myeloid sarcoma of urinary bladder is a rare disorder. We report a 71-year old man with hematuria. He was also under follow up for unclassified myeloproliferative/ myelodysplastic disorder, diagnosed 2 months ago. Abdominal ultrasonography and computed tomography findings were normal. Diagnostic cystoscopy revealed patchy areas of mucosal swelling with hyperemia. Histopathological examination of biopsies demonstrated a neoplasm composed of blasts showing myeloperoxidease positivity by immunohistochemistry. To our knowledge, current case is the first case of myeloid sarcoma without evidence of a mass lesion in the urinary bladder with a concurrent diagnosis of myelodysplastic /myeloproliferative disease, unclassifiable.

The significance of Galectin-3 expression in the immunocytochemical evaluation of thyroid fine needle aspiration cytology.

The aim of this study is to evaluate the significance of immunohistochemical expression of Galectin-3 in the differential diagnosis of benign and malignant thyroid nodules. We studied the fine needle aspiration specimens of 38 patients who had evaluated for nodular goiter and undergone a thyroid surgery between 2004-2005. Slides had been stained immunocytochemically with Galectin-3. The cytoplasmic staining of Galectin-3 was analyzed. Three cases of five follicular carcinomas had positive staining for Galectin-3, while two had not. Two cases with follicular adenomas were negative for Galectin-3. Five cases of six papillary carcinomas had positive staining for Galectin-3, while one case (the case with a papillary microcarcinoma) had not. The single cases with medullary and anaplastic carcinomas were negative for Galectin-3. None of the cases with a benign thyroid pathology had positive staining for Galectin-3. Galectin-3 immunocytochemical staining, had a sensitivity of 61.5%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 83.3% for thyroid malignancies. For the evaluation of follicular neoplasm, Galectin-3 immunocytochemical staining had a sensitivity of 60%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 50%. Galectin-3 expression in thyrocytes is a strong indicator of a malignant proliferative lesion especially for papillary and to an extent in follicular thyroid neoplasms. Galectin-3 could be used as a supplementary marker for cytological diagnosis.