Yesim Ertan

Dural plasmacytoma mimicking meningioma in a patient with multiple myeloma

Apart from calvarial infiltration, intracranial involvement in multiple myeloma is uncommon. Diffuse leptomeningeal invasion with or without parenchymal involvement is most common. Dural infiltration without involvement of the parenchyma, leptomeninges or skull is rare. The differential diagnosis of a dural plasmacytoma includes meningioma, which has a similar MRI appearance, metastasis, lymphoma and sarcoma of the dura mater. We present a patient with multiple myeloma presenting with an intracerebral mass mimicking a meningioma on MRI. Multiple myeloma had been diagnosed seven years previously. The patient presented with headache and speech disturbance 12 months after autologous peripheral stem cell transplantation for recurrence of multiple myeloma. MRI revealed a left temporal extra-axial mass with a dural tail mimicking meningioma. Histopathological examination of the mass after excision showed multiple myeloma immunopositive for IgG, kappa light chain and CD38. There was no recurrence after postoperative radiotherapy. Plasmacytoma should be considered in the differential diagnosis of a solitary dural mass, particularly in a patient with multiple myeloma.

Multiple spinal metastases of cranial gliosarcoma: a case report and review of the literature.

Gliosarcoma is a rare brain tumor that consists of both glial and mesenchymal components. We report the case of a 68-year-old female with cranial gliosarcoma metastatic to the spinal cord. Initially, the patient was diagnosed with cranial gliosarcoma and treated with surgical resection followed by radiotherapy. Four months after she completed treatment, she presented with a sudden onset of hemiplegia. MRI (Magnetic Resonance Imaging) scan demonstrated two masses at the thoracic spinal cord. Immediate surgery was performed and the lesions were resected. No further therapy was recommended due to the poor condition of the patient. The patient subsequently died 3 months after diagnosis of the spinal cord metastases. There are about 20 reported cases of metastatic gliosarcoma and most focus on systemic metastases of gliosarcoma. Spinal cord metastases are, however, very rare and here we report such a case. Available literature on metastatic gliosarcoma was also reviewed.

Rapid transformation of atypical myeloproliferative disorder with consistent t(8;13) to B-cell acute lymphoblastic leukemia: A case report

Abstract 8p11 myeloproliferative syndrome (EMS; also known as the stem cell leukemia syndrome-SCLL) is a rare atypical myeloproliferative disorder associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand independent FGFR activity. The most commonly observed translocation of this syndrome is t(8;13), which results in the expression of a chimeric ZNF198-FGFR1 tyrosine kinase. Disease phenotype associated with this translocation has some typical features such as poor prognosis, and transformation to mainly acute leukemia and non-Hodgkin lymphoma; commonly with a T-cell phenotype in which obtaining and maintenance of remission is difficult by conventional chemotherapy. We hereby present a case diagnosed as atypical chronic myeloproliferative disease with consistent t(8;13)(p12;q12) and transformed rapidly to pre-B-cell acute lymphoblastic leukemia which is a rare clinical presentation.

Can CD10 be used as a diagnostic marker in thyroid pathology

CD10–common acute lymphoblastic leukemia antigen is a membrane-bound zinc metalloproteinase that is expressed by different hematopoietic cell types at unique stages of lymphoid and myeloid differentiation. It was reported to be expressed in various nonlymphoid cells and tissue, as well as in various types of neoplasms. Recently, it has been found to be useful in the differential diagnosis of benign and malignant follicular-patterned lesions of the thyroid. In the present study, we evaluated the staining pattern of CD10 in various thyroid lesions, including 14 benign and 61 malignant cases, as well as in adjacent thyroid tissue. CD10 was negative in normal thyroid tissue, adenomatous nodules, minimally invasive follicular carcinoma, and well-differentiated carcinoma. It was expressed in nine of 14 (64.2%) conventional papillary carcinomas, four of 24 (16.6%) follicular variant of papillary carcinomas, three of six (50%) papillary microcarcinomas, one of nine (11.1%) widely invasive follicular carcinomas, and three of ten (30%) follicular adenomas. In contrast to results of previous studies, CD10 is not useful in the classification of thyroid follicular lesions as benign or malignant, but it shows strong positivity in conventional papillary carcinoma.

Intrahepatic cholangiocarcinoma arising in chronic viral hepatitis-associated cirrhosis: two transplant cases.

Hepatitis C virus (HCV) or hepatitis B virus (HBV)-related cirrhosis is known to be a risk factor for hepatocellular carcinoma (HCC). Recently, these viruses have been reported to have an etiologic role in the development of intrahepatic cholangiocarcinoma (ICC). Herein we have reported two cases of HCV- and HBV-related cirrhosis with ICC in whom the pretransplant diagnosis was HCC. The patient with HCV cirrhosis, was a 47-year-old woman with a large nodule in the right lobe. The patient with HBV cirrhosis was a 45-year-old man with two nodules. Serum tumor marker levels, carcinoembryonic antigen (CEA), alphafetoprotein (AFP), and carbohydrate antigen 19-9 (CA 19-9) were determined before live donor liver transplantation (LDLT). The patient with HCV cirrhosis showed mildly elevated serum levels of AFP. The patient with HBV cirrhosis showed an elevated CA 19-9 level. On microscopic examination, all nodules exhibited typical morphological findings of adenocarcinoma. The patient with HCV cirrhosis developed brain metastases 4 years after LDLT. The patient with HBV cirrhosis is disease-free at 18 months after transplantation. In cirrhotic patients with active malignancy who are candidates for LDLT, ICC should be considered in the differential diagnosis. Although the literature is limited, selected patients with ICC may benefit from LDLT.

A novel disease-causing CD40L mutation reduces expression of CD40 ligand, but preserves CD40 binding capacity

Mutations in CD40 ligand (CD40L) that permit residual CD40L expression typically impair binding of CD40. We report a male patient who presented with recurrent bacterial respiratory tract infections, normal IgM, decreased IgG, absent IgA levels, and CD40L expression at ~50% of the level observed in the normal control. He subsequently developed autoimmunity, inflammatory bowel disease, severe opportunistic infections suggestive of a combined immunodeficiency, and a cervical spine schwannoma. Whole exome sequencing of the patients genomic DNA revealed a novel missense mutation (p.H47Y) in CD40L. Although this mutation was predicted to be benign in silico, flow cytometry at 13 years of age demonstrated markedly decreased CD40L expression (~32% of normal control) that retained the capacity to bind soluble CD40-Ig, suggesting that the mutation impairs CD40L surface expression without affecting its affinity for CD40. This case highlights the variability in the clinical evolution and phenotype of CD40L deficiency.

Primary glioblastoma of the Medulla Spinalis: A Report of Three Cases and Review of the literature

Primary spinal glioblastoma multiforme (spinal GBM) is not a very common entity. This paper presents an outline of this rare neoplasm, its clinical presentation, course, management and outcome and reports a 3-case series of spinal GBM. In this 3-case series with spinal GBM, one of the patients was operated for hydrocephalous 10 months later following the tumor surgery and another patient had cerebral metastasis after the surgery. In the postoperative period, two of the cases received radiotherapy and one received combined radiotherapy and chemotherapy with steroid therapy together following the tumor surgery. The review of the pertinent literature has revealed that due to the scarcity of the reported cases of primary spinal GBMs, this issue requires a closer look. GBM behaves more aggressive in medulla spinalis than it behaves when it originates from cerebrum. It may disseminate to the cerebrum during its course and it may cause hydrocephalus due to this dissemination (metastasis).

Tiroid papiller mikrokarsinomlarında multisentrisite

Tiroid kanseri, endokrin organ kaynakli kanserler arasinda en sik gorulenidir (1,2). Tiroid kanserlerinin yaklasik %85’ini papiller tiroid kanseri olusturmaktadir. Papiller kanserlerin bir alt grubu olarak siniflandirilan ve ≤10mm tumorler olan ‘papiller mikrokarsinomlar’ tum papiller kanserlerin %30’unu olusturmakta ve daha az agresif davrandiklari kabul edilmektedir (2-5). Yuksek sagkalim oranlarina ragmen bazi hastalarda lokal nuks ve metastazlar ortaya cikmakta ve bu durum daha agresif bir cerrahi tedaviyi gerektirebilmektedir. Her ne kadar literaturde tanimlamasi konusunda kargasa mevcut ise de ‘tiroid kanserinde multisentrisite’, tiroid kanserinin, ayni tiroid lobunda veya iki lob icerisinde birden fazla yerde bulunmasi olarak tanimlanir (6). Gunumuzde yaygin olarak kullanilan tani yontemleri ile papiller mikrokarsinom tanisini ve multisentrik ozelligini belirlemek kolay degildir. Mikrokarsinomlar genellikle palpe edilemez ve klinik olarak sessiz seyretmektedir. Otopsi calismalarinda papiller mikrokarsinom gorulme orani bolgesel olarak degismekle birlikte %0.01-%35 arasinda bildirilmektedir ve bu oranlar cogu papiller mikroarsinomun benign davranisli oldugunu desteklemektedir (2,3,7,8). Cogu mikrokarsinom tanisi ise ‘tesadufi’ olarak benign davranisli tiroid hastaligi nedeniyle ameliyat edilen hastanin piyesinin patolojik incelemesi sonucu ortaya cikmaktadir. Gunumuzde daha yuksek cozunurluklu ultrasonografi ve ultrasonografide kusku duyulan, capi 1 cm’den kucuk nodullere uygulaARAŞTIRMA YAZISI

Variable sonographic spectrum of parathyroid adenoma with a novel ultrasound finding: dual concentric echo sign.

AIMS To review the detailed gray-scale and Doppler ultrasonography features of histologically proven parathyroid adenomas (PAs) evaluated with high-end ultrasonography devices and to present a novel ultrasonography finding called the dual concentric echo sign in PA with histopathologic correlation which was encountered during detailed analysis. MATERIAL AND METHODS Fifty-six PAs with histopathological result were enrolled. The longest dimension, shape, distance to skin surface, internal echo and Doppler US features obtained with high-end US devices were evaluated. RESULTS PAs had variable range of shape including oval, irregular, fusiform, lobulated, crescent-shaped, and nodular configuration. In nine patients the lesions were shown to have cystic components and calcifications were seen in four cases. Dual concentric echo sign was detected in 18% PAs. Histological reevaluation of this subgroup demonstrated significantly increased edema (p<0.01), and ectatic vessels (p=0.02) in the central part of the lesion compared to the rest of the PAs. CONCLUSIONS The results of the study led to the conclusion that PAs have variable gray-scale and Doppler findings. Typical sonographic features like ovoid shape, homogeneously hypoechoic pattern may not be present in all PAs. Dual concentric echo sign which is a novel sonographic pattern may be suggestive of a PA.

Intraoperative parathyroid hormone monitoring corroborates the success of parathyroidectomy in children.

Objective: To assess the efficacy of intraoperative parathyroid hormone (PTH) monitoring in evaluating the outcome of parathyroidectomy in pediatric patients. Methods: Intraoperative PTH monitoring during parathyroidectomy was performed in five children (3M, 2F); three had parathyroid adenomas (single gland disease) and two had primary hyperplasia. One patient had undergone two previous surgical interventions to remove the parathyroid glands, but the PTH levels had remained high with persistence of symptoms. Immunoradiometric analysis was used for PTH measurements. Preoperative PTH values were obtained to monitor the baseline levels. Serum samples were collected 20 minutes after removal of the adenoma/parathyroid gland(s) and PTH levels were compared with preoperative values. Specimens were also confirmed by frozen sectional examination. Results: Mean age of the patients was 11 years (range: 3 months-16 years). Mean preoperative PTH values were 633.3±579 pg/mL (range: 143-1300 pg/mL). Intraoperative values decreased to 18.7±5.5 pg/mL (range: 8-27 pg/mL) following removal of the gland(s). Normal calcium levels were achieved with adequate management following surgery. One patient (with multiple surgeries and found to have an ectopic parathyroid gland) had hungry bone syndrome after the operation and was treated successfully. There were no major complications. All patients maintained normal calcium/phosphorus levels in the follow-up period, ranging from 2 to 5 years. Conclusion: An ectopic parathyroid gland or another undetected adenoma can be overlooked during surgery. Owing to the short life of the hormone, intraoperative PTH monitoring to determine PTH clearance proved to be a feasible marker for adequacy and safety of surgery and “cure”.