Atypical adult-onset subacute sclerosing panencephalitis.

Although subacute sclerosing panencephalitis is almost exclusively a childhood disease, it can occur in adults as well. We present an atypical case of adult-onset subacute sclerosing panencephalitis. The disease was characterized by prolonged insidious course followed by accelerated and aggressive phase, atypical EEG findings, and absence of myoclonic jerks. The diagnostic and treatment-related pitfalls are discussed.

Primary malignant fibrous histiocytoma of the spleen: recurrence eight years after splenectomy--report of a case and literature review.

Primary intraabdominal malignant mesenchymal tumors are very rare. There are just few cases of intraabdominal visceral malignant fibrous histiocytoma in the literature. We report a case of primary malignant fibrous histiocytoma of the spleen in a 57-year-old man, with a recurrence eight years after the splenectomy. After the initial surgery the patient was without complaints, and refused to receive chemotherapy or radiotherapy. Eight years after the surgery the patient reported due to general weakness and malaise when the diagnosis of disease relapse was established. Radical surgery was performed although the tumor involved large curvature of the stomach, left crus of the diaphragm, splenic flexure of the colon and tail of pancreas. Four months after the surgery patient died. To the best of our knowledge, to date, only 18 cases have been reported in the literature, describing malignant fibrous histiocytoma of the spleen.

Paraganglioma of the urinary bladder--clinicopathological, immunohistochemical and electron microscopy analysis--a case report.

Tumors that grow within the adrenal medulla are called pheochromocytoma; when located extra-adrenal, they are called paraganglioma. Paraganglioma of the bladder are very rare, with only 180 reported cases. Less than 30 were malignant. We report a case of a 72-years old man with bladder paraganglioma who presented with painless hematuria. Urgent transurethral resection (TUR) was performed. Definitive pathohistological diagnosis was confirmed to imunohistochemical and electron microscopy. Clinical diagnostic showed normal value of epinephrine and norepinehrine in the urine. Scintigraphy of entire body and targeted pictures of pelvis where taken 24, 48 and 72 hours after administration of RI. No loci of pathologic accumulation of 131-I MIBG where found. Computer tomography (CT) of pelvis and abdomen were normal. Considering staging and pathohistological analysis, we treated our patient with TUR and longtime follow-up afterworth.

Tumor and breast volume ratio as a predictive factor for axillary lymph node metastases in T1c ductal invasive breast cancer: prospective observational clinico-pathological study.

OBJECTIVE: The status of the axilla is the single most important prognostic indicator of overall survival in patients with breast cancer. Sentinel-node biopsy has false-negative rates of 5-10%. The aim of this study was to assess the importance of tumor and breast volume ratio as a predictive factor for axillary lymph node metastases in patients with T1c ductal invasive breast cancer. METHODS: This study included 136 consecutive patients with T1c ductal invasive breast cancer. Three tumor diameters were measured. Tumor volume was calculated by the formula for ellipse. Breast volume was measured preoperatively. Tumor and breast volume ratio was calculated and shown per thousand. RESULTS: Tumor and breast volume ratio is a new independent predictive factor for axillary lymph node metastase in T1c ductal invasive breast cancer. CONCLUSIONS: This predictive factor could help to define a subgroup of patients who will be at a higher risk for axillary lymph node metastase and would benefit from additional close follow up or axillary lymph node dissection.

Extracranial propagation of glioblastoma with extension to pterygomaxillar fossa.

BACKGROUND: Glioblastoma multiforme is a highly malignant primary brain tumor that shows marked local aggressiveness, but extracranial spread is not a common occurrence. We present an unusual case of recurrent glioblastoma in 54-year old male that spread through the scull base to the ethmoid and sphenoid sinuses, to the orbita, pterygomaxillar fossa, and to the neck. METHODS: A 54-year old male underwent left temporal resection because of brain tumor of his left temporal lobe. Operation was followed by external beam radiation combined with temozolomide. The tumor recurred eight months after first surgery. The patient developed swelling of left temporal region, difficult swallowing and headache. MRI of head showed recurrent tumor, which invaded orbita, ethmoid and sphenoid sinuses, nasal cavity, pterygomaxillar fossa. RESULTS: The patient died ten months after initial diagnosis of glioblastoma multiforme, and two months after his second operation. CONCLUSIONS: The aggressive surgical operation helped to downsize the tumor mass as much as possible, but did not prolonged significantly the life or improved the life quality of the patient. The current literature is reviewed, and the diagnostic approaches as well as therapeutic options are discussed.

[Trends in stage and age characteristics of the female breast cancer treated in 11-year period at University Hospital "Dubrava"]. / Analiza dobne distribucije i stupnja lokalne prosirenosti raka dojke kod bolesnica operiranih u 11-godisnjem razdoblju u Klinickoj bolnici "Dubrava".

In a retrospective study we analyzed and compared the factors of local spread of breast cancer, namely tumor size and status of regional lymph nodes and patient age in the period from 1997 to 2007. The study includes only those patients in whom the diagnosis was made in an open surgical biopsy and it was possible to determine the local extent of disease at the time of diagnosis (pT and pN stage), a total of 1202 patients. We also compared the findings in patients younger and older than 50 years. The average tumor size at diagnosis was 2.2 cm and ranged between 1.7 cm (2005) and 2.4 cm (1997, 1999). The percentage of metastases in regional lymph nodes varied between 37% (2001) and 50% (2000 and 2006). The average of 6% of all cases of breast cancer was diagnosed in non-invasive (in situ) stage. The mean patient age was 56.8 years and for the observed age ranged between 54.3 (1999) and 59.1 (2004) years. There was no statistically significant difference in relation to disease stage or patient age at diagnosis observed throughout the entire period of time. There was also no statistical difference in stage of disease at the time of diagnosis in patients younger and older than 50 years.

Fine needle aspiration cytology of metastatic Merkel cell carcinoma.

Merkel cell carcinoma (MCC) is uncommon cutaneous malignant neuroendocrine tumour of the elderly people with rapidly growing skin nodules found frequently on sun-exposed areas of the body. MCC is often an aggressive tumour with high tendency for local recurrence, lymph node involvement and distant metastases. This paper reports a case of metastatic MCC diagnosed by fine needle aspiration cytology (FNAC), flow cytometric deoxiribonucleated acid (DNA) analysis, pathohistology and electron microscopy. The cytological features in aspirate (stained with Papenheim and Papanicolaou staining) included increased cellularity, discohesive groups of small-to-medium size malignant cells with uniform, round-to-oval nuclei with moulding effect, fine chromatin, multiple micronucleoli and scanty cytoplasm. DNA flow cytometric analysis of the aspirate showed unexpected results for clinically aggressive behaviour of this tumour (the patient died after 21 months), and revealed that tumour contained diploid peak with DNA index of 1.1. The proliferation was high with elevated S-phase fraction (21%). The cytological diagnosis of possible metastatic MCC was confirmed by histological one as well as by electron microscopy presented the pathognomonic features for this tumour: dense-core neurosecretory granules with diameter of 100-250 nm surrounded by whorls of intermediate filaments. MCC provides an enormous challenge for the morphologist because of a wide range of differential diagnosis and for the clinician because this tumour has a highly malignant potential for local recurrence, nodal and distant spread and very often is combined with other tumours. Therefore it is important to perform FNAC of different lesions in the same patient because it can distinguish MCC from the other tumours.

Intraoperative imprint cytological assessment of the subareolar tissue of the nipple areola complex (NAC).

One of the criteria of selection for skin sparing mastectomy (SSM) with nipple areola complex (NAC) preservation is to exclude the neoplastic involvement of subareolar tissue (NAC base) in order to minimize the possibility of local recurrence. The most common way to assess the possible neoplastic involvement is intraoperative frozen section of the NAC base tissue. Because of its limitations, particularly the false negative results due to unsampling, we tried to use intraoperative imprint cytology for more thorough intraoperative assessment. The aim was to compare intraoperative imprint findings with the definitive histology of the NAC base, to evaluate diagnostic accuracy of this method and possibility to substitute frozen section for intraoperative assessment of NAC base. A prospective clinical study was conducted of 208 consecutive female patients who underwent open biopsy because of carcinoma. Intraoperative imprints were taken from the excised subareolar tissue which was then routinely processed for definitive histology. Imprint findings designated positive, negative, suspicious or atypia, were compared with definitive histological findings. Our results with 7.5% false negative rate, 9.8% false positive rate, sensitivity of 50% and specificity of 87.58% argue that imprint cytology might not be sufficient as an exclusive method for the intraoperative assessment of the NAC base though it should be used routinely in conjunction with frozen section examination.

Misleading presentations of malignant breast diseases--role of clinical cytology.

We described two examples with misleading presentations to draw attention to the role of clinical cytology as apart of multidisciplinary approach to breast lesions. In the first case--Paget's disease of the nipple, there was no obvious clinical and radiological evidence of breast cancer, while the second case--primary non-Hodgkin lymphoma of the breast imitated advanced breast carcinoma. The question is whether accurate and fast diagnoses can be made without cytological examinations. It must be kept in mind that first-hand clinical information and contact with the patient is necessary in rendering accurate cytological diagnoses.

Cerebellar glioblastomas: pathophysiology, clinical presentation and management.

Glioblastoma multiforme usually affects the cerebral hemispheres with the peak age of onset in the sixth or seventh decade, while cerebellar glioblastoma multiforme is a rare tumour especially in younger patients. Most result from de-differentiation from low grade astrocytoma (secondary glioblastoma) or can develop de novo (primary glioblastoma). Primary glioblastomas develop in older patients while secondary glioblastomas develop in younger patients and contain TP53 mutations as the earliest detectable change. We report a 28 year old patient with primary multi-focal cerebellar glioblastoma multiforme and review the pathophysiology, clinical presentation, diagnosis and treatment of cerebellar glioblastomas.

Sinus pericranii in the left frontal region involving the superior eyelid: a case report.

BACKGROUND: Sinus pericranii is a rare asymptomatic communication between the intracranial and extracranial venous drainage pathways. The venous flow in this condition circulates through abnormal dilated veins in both directions. PATIENT/METHODS: We describe an unusual location of an accessory sinus pericranii that involved the left frontal bone along with the superior orbital rim and the upper eyelid, with special focus on therapy. RESULTS: The patient did not have any complication during the first and second surgery. Final outcome was excellent. The patient did not show any evidence of disease recurrence 1 year after the surgery. CONCLUSION: We propose a multidisciplinary approach in the treatment of such lesions with a two-step surgery. Excision of the sinus pericranii is possible if the sinus pericranii is not a major venous outflow channel of the brain, which can be evaluated by angiography.

Unrecognized hemangiopericytoma of posterior cervical region with intracranial extension.

Hemangiopericytoma of the posterior cervical space and occipital bone is an uncommon lesion which should be considered in the differential diagnosis of a lumpy and highly vascular lesion of the posterior cervical space. We report the case of a 47-year-old woman who experienced sudden and painful occipital and posterior cervical swelling. She underwent a blind biopsy which was complicated by profuse bleeding. The palpable lesion was not properly diagnosed preoperatively, and the endocranial extension of the lesion was overlooked by her surgeon who performed a blind biopsy without adequate diagnostic imaging who inadvertently invaded the posterior cranial fossa during biopsy. We would like to emphasize the need for appropriate non-invasive diagnostic investigations before any biopsy of head and neck lesions that may extend deeply.

Intracranial recurrence and distant metastasis of scalp dermatofibrosarcoma protuberans.

Only few cases of scalp dermatofibrosarcoma protuberans with intracranial and distant metastasis have been reported. Here we report a case of scalp dermatofibrosarcoma protuberans with frequent local recurrence, intracranial invasion and with distant lung metastasis during 6 years of treatment. We would like to emphasize difficulties in surgical treatment of such invasive and locally recurrent tumors of scalp, and necessity to understand new molecular pathogenesis of dermatofibrosarcoma protuberans and potential treatment strategy with imatinib for patients with surgically untreatable disease. Close surveillance of patients with scalp dermatofibrosarcoma is necessary due recurrence nature of tumor.

Nipple-areola complex preservation: predictive factors of neoplastic nipple-areola complex invasion.

Recently, skin-sparing mastectomy (SSM) with nipple-areola complex (NAC) preservation has been promoted as an oncologically safe procedure in practice for selected patients. The criteria of selection have not been yet defined precisely. The focus of this study was to investigate predictive factors of NAC-base neoplastic involvement to define the indications for NAC preservation. A prospective clinical study was conducted of 108 randomly selected female patients with invasive breast cancer. Analyzed markers of NAC involvement were tumor-nipple distance (TND), tumor size, localization, histologic type, grade, lymphovascular invasion (LVI), site, and axillary lymph-node status. The definitive histologic findings of the NAC base were compared with analyzed markers and the frozen section results. NAC base was positive in 23.15% patients at definitive histology with false-negative results in 4.63% patients at intraoperative frozen section. Significant differences were found in TND, tumor size, axillary lymph-node status, and LVI. There were no significant differences in tumor grade and site and not enough cases for statistical evaluation in histologic type and localization. Clinical indications for NAC preservation, according to this study, include tumors < or =2.5 cm, TND >4 cm, negative axillary lymph node status, and no LVI. Considering the possibility of pre- or intraoperative measurement, tumor size, and TND evaluation will result in the lowest possible mistakes in NAC preservation. Frozen section analyses of the NAC base, because of the "false-negative" possibility, could be deemed as a relative prognostic factor until definitive histologic findings. The presence of an extensive intraductal component (EIC) in the "borderline" cases of these criteria could be an additional argument for NAC removal.