Primary Left Atrial Angiosarcoma Presenting as Acute Coronary Syndrome.

Primary cardiac neoplasms are extremely rare and often overlooked as differential diagnosis. Angiosarcomas are the most common primary malignant neoplasms of the heart often with nonspecific symptoms. We present a 43-year-old woman admitted to our hospital with chest pain and inferoposterolateral myocardial infarction. Coronary angiography indicated the distal occlusion of the left circumflex artery. Transthoracic and transoesophagic echocardiography revealed a mass in the left atrium with probable myocardial infiltration and vascularisation. The mass in the left atrium was removed by surgical resection, and histopathology confirmed angiosarcoma. We emphasize the pivotal role of transthoracic and transoesophageal echocardiography in evaluating even rare differential diagnosis of acute coronary syndrome as cardiac neoplasms.

ANGIOMYOMA OF THE UPPER LIP - CASE REPORT AND REVIEW OF THE LITERATURE.

A case is presented of upper lip angiomyoma in a 36-year-old man. The tumor was painless, palpable and clinically visible. The operation was performed under local anesthesia in the Outpatient Department. There were no complications during the postoperative course. Current literature on the subject is listed in the introduction, followed by presentation of the case and histologic characteristics of the tumor. This case is described as one of the differential diagnostic possibilities in the diagnosis of soft tissue tumors of the lip. Also, through review of the literature, different clinical appearances of angiomyoma, histologic variations and immunohistochemical characteristics are discussed that can help identify this tumor.

Erlotinib for coexisting typical bronchial carcinoid and advanced lung adenocarcinoma: does the epidermal growth factor receptor mutation status matter?

Adenocarcinoma (AC) is the most common type of primary pulmonary malignancy. Lung carcinoid, however, is a rare neuroendocrine tumor. Their coexistence is extremely uncommon. We report the unique case of synchronous advanced lung AC of the right upper lobe (stage IIIB) and typical endobronchial carcinoid tumor in the contralateral lower lobe in a 49-year-old white female who had never smoked. PET-computed tomography scan revealed a fluorine-18-fluorodeoxyglucose-avid AC lesion, whereas the carcinoid tumor was fluorine-18-fluorodeoxyglucose occult. After two lines of platinum-based combination chemotherapies and radiotherapy, the AC progressed, and oral tyrosine kinase inhibitor therapy with erlotinib was initiated in third line. On erlotinib, the AC remained stable for 50 months until disease progression, whereas the carcinoid completely regressed. Molecular testing of the rebronchoscopied AC revealed an exon 19 deletion mutation in the epidermal growth factor receptor (EGFR) gene, whereas the carcinoid was retrospectively EGFR mutation negative. The patient eventually succumbed to ileus caused by intra-abdominal spread of disease, surviving a remarkable 80 months with good performance status throughout most of the follow-up period. To the best of our knowledge, this is the first reported case of synchronous primary lung cancers with different EGFR mutation status, describing an unexpected response of an EGFR-wild-type carcinoid to third-line erlotinib.

Dilemma in clinical diagnosis of right ventricular masses.

Detection of an intracardiac mass always represents a clinical challenge. We present a 61-year-old female patient with symptoms of New York Heart Association class III. Two-dimensional transthoracic echocardiography revealed a hypoechogenic mass in the cavity of the dilated right ventricle (RV). Cardiac MRI described a pathologic structure of the RV free wall with pedunculated tumor in its cavity. Three months later, on a repeated echocardiography, there were three individual masses. The patient underwent surgery and the pathohistologic report demonstrated thrombotic masses. During the postoperative period, after reviewing all medical records, the conclusion was arrhythmogenic RV cardiomyopathy. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:362-369, 2017.

Botryoid Cyst, a Rare Type of Odontogenic Cyst

Botryoid cyst, a rare type of odontogenic cyst, is usually considered a multilocular variant of lateral periodontal cyst. It was first described in 1973, and up to the present less than a hundred cases have been reported in the literature. Botryoid cysts are characterized by a multilocular histologic finding and a higher risk of recurrence than lateral periodontal cysts. In this article, we present a case of botryoid cyst from our clinical practice, accompanied by literature review of previous cases reported in the literature so far. Study results showed that botryoid cysts mostly affected population in their fifties, with a slight female (53.5%) predominance over males (46.5%). The mandible was the most frequently affected region, accounting for 70 (83.3%) cases. Maxilla was only sporadically affected accounting for 14 (16.7%) cases. Radiographic appearance of botryoid cysts can be multilocular or unilocular. It is concluded that the recurrence rate is relatively high (30.1%), therefore long term follow up is needed.

The Role of Standard Echocardiographic Parameters in Endomyocardial Biopsy Proven Cardiac Amyloidosis.

Primary light-chain (AL) amyloidosis is a plasma cell dyscrasia associated with the deposition of immunoglobulin-derived amyloid in multiple organs. In the heart, this results in an infiltrative cardiomyopathy, with increased left ventricular wall thickness, normal or decreased left ventricular (LV) cavity size and congestive heart failure. Cardiac involvement is a major determinant of prognosis of AL amyloidosis. We report a patient with cardiac amyloidosis proven by cardiac biopsy, and aim to point out at transthoracic echocardiography as the hallmark of diagnostics. Echocardiography revealed increased LV thickness at 20mm, impaired LV ejection fraction (EF) at 35%, enlarged atria, transmitral deceleration time at 156 ms and increased E/A ratio at 4.25. Early diagnosis and intervention can have a significant impact on the patient's response to treatment, especially when the underlying condition involves a malignancy or infiltrative disorder. Standard transthoracic echocardiography as a noninvasive diagnostic tool is valuable and has a significant role in diagnosis and prognosis.

Calcifying epithelial odontogenic tumor of the maxilla (Pindborg tumor).

Calcifying epithelial odontogenic tumor (CEOT), or the Pindborg tumor, is very rare neoplasm, which accounts up to 1% of all odontogenic tumors. These tumors involve mandible almost twice as common as the maxillary bone, mostly in the premolar and molar region and present at first with local swelling. There is no gender predilection and the tumor usually appears between 2nd and 6th decade of life. We report the case of a 36-year-old male patient with a Pindborg tumor in the maxillary region on the right side, also involving the adjacent maxillary sinus, with destroying of the local anatomical structures. Complete surgical excision of the tumor has been performed and four years after surgical treatment, there is no sign of recurrence.

Pleomorphic adenoma in ectopic salivary gland tissue in the neck.

A case of pleomorphic adenoma originating from ectopic salivary gland tissue (ESGT) of the upper neck is reported. A 34-year-old male patient was referred to our Department for a painless swelling in the right submandibular region. Preoperative evaluation (clinical examination, fine-needle aspiration cytology (FNAC) and imaging studies) was performed and the finding was that of pleomorphic adenoma in ESGT A modified "S" incision with extension to the submandibular region was performed and the tumour was extirpated. The histopathological report confirmed our initial diagnosis. No recurrence was obtained during a four-year follow-up period. Isolated neck mass may be overlooked as ectopic salivary gland tissue neoplasm (ESGTN). Proper preoperative assessment and optimal surgical treatment are the keys for successful management of these rare tumours. The distinction between metastatic lesion from a head and neck tumour and ESGTN may present considerable diagnostic problem. A review of the literature on ESGT and associated tumours with emphasis on clinical features, diagnosis and treatment is also presented.

Inflammatory pseudotumor presenting as a facial swelling.

We present a case of inflammatory pseudotumor (IPT) presenting as a facial swelling after an accidental hit on a right side of a face. As swelling did not resolve, dental examination and teeth extraction were done by a dentist presuming the swelling was misdiagnosed with infection of dental origin. Swelling grew even bigger and patient was referred to Department of Maxillofacial Surgery. CT scan of the face and FNA of the lesion was ordered. A homogenous tumor mass in the right infraorbital region in front of anterior wall of the maxillary sinus was seen on CT The result of the FNA was reactive hyperplasia of the lymph node. Since the lesion was easily accessible surgical exploration and complete extirpation was done. Pathohistological analysis indicated a low grade B-cell Non Hodgkin lymphoma. PCR showed policlonality of B cells discarding the diagnosis of lymphoma. Pathohistological review showed diffuse intramuscular, perineural and perivascular infiltration with small lymphocytes without formation of germinative centers. Imunohistochemistry was positive for CD20 and CD3. Taking into account all features the diagnosis of IPT was established. Diagnosis of IPT is a diagnosis by exclusion, combining clinical, radiological and pathohistological characteristics. Lack of clear histologic criteria makes differential diagnosis extremely difficult. Our case is unique regarding localisation of head & neck IPT no case presenting on the face in infraorbital region has been described in the literature. Although IPT is very rare in general and especially on the face, one should be aware of it when considering differential diagnosis of facial swelling.

The importance of team work of cytologist and surgeon in preoperative diagnosis of intraoral minor salivary gland tumours.

Tumours arising from oral minor salivary glands may exhibit an overlap of clinical and morphological features that may produce diagnostic and therapeutic dilemmas. The aim of this study is to asses the value of fine needle aspiration cytology (FNAC) in differentiation of benign and malignant tumours and to render a specific diagnosis. We evaluated the team work of surgeon and cytologist to improve diagnostic accuracy. Two steps are important for accuracy: sampling aspirate that should be done together by surgeon and cytologist and cytological microscopic analysis of the smears that should be performed by an experienced cytologist. The study included 132 patients with intraoral minor salivary gland tumours between 2002 and 2011. Adequate material was obtained from 121 (91.7%) patients. FNAC was usually performed by cytologist in a team with maxillofacial surgeon at cytology department that is more convenient for preparing the samples and especially for ROSE procedure (rapid-on site evaluation) of smears. In such a way the cytologist checked the adequacy of samples and decided whether some ancillary techniques should be used and therefore repeat FNAC. A total of 82 patients underwent surgery, 40 with malignant and 42 with benign tumours. Preoperative cytological diagnoses were compared with histopathological ones using histopathology as a gold standard. The most common benign tumour was pleomorphic adenoma and among malignant tumours adenoid cystic carcinoma. The most commonly affected site was the palate. The team work of surgeon and cytologist achieved specificity of 95.1%, sensitivity of 97.6% and diagnostic accuracy of 96.3%. We can conclude that although subclassification of some tumour types of salivary glands remains poor, FNAC is invaluable in patient triage and therefore should be considered in the first line investigations of these lesions by the cytologist and surgeon.

Lifestyle habits and the risk of head and neck cancer in Zagreb region.

In 2002, International Agency for Research on Cancer launched a multicentric case-control study of head and neck cancer in 15 centres in 11 European countries, Croatia included. The Alcohol-related Cancers and Genetic Susceptibility in Europe (ARCAGE) study database with approximately 2500 case-control pairs is currently the largest head and neck cancer patients database in Europe. The aim of the present study was to estimate the risk of smoking and alcohol consumption in head and neck cancer patients from Zagreb region recruited in scope of the ARCAGE study. The cases (45 males, 9 females) and the controls (37 males, 9 females) were recruited from University Hospital Dubrava and Merkur University Hospital. A blood sample and a lifestyle questionnaire were collected from all study subjects. Data were analysed by logistic regression analysis. The distribution of cases was: 43 oral, 9 pharyngeal and 2 laryngeal cancer cases. Ever-smokers had a 4-fold higher cancer risk (adjusted OR 4.3 (CI 1.20-15.4)) compared to never-smokers. The adjusted odds ratio for alcohol consumption more than once a week was 4.1 (CI 1.5-11.2). The results are in keeping with previously published data from other countries.

Angiomyoma--angioleiomyoma of the cheek.

The authors present the case of an angiomyoma--angioleiomyoma of the cheek in a 58-year-old man. The tumour was palpable, although clinically not visible, and the only case of a tumour of smooth muscle treated in the Clinical Department of Oral Surgery over the last 40 years. The operation was performed in the Outpatient Department by intraoral procedure. The postoperative course passed without complications. Current literature on leiomyomas is cited in the Introduction, followed by presentation of the case and histological characteristics of the tumour The example is presented as a rarity and one of the differential diagnostic possibilities in the diagnostics of soft tissue tumours in the oral cavity.

Large erupting complex odontoma in a dentigerous cyst removed by a piecemeal resection.

Odontomas (ODs) are the most frequent odontogenic tumors in Western societies, they are often asymptomatic and discovered during routine radiography The purpose of this report was to describe the case of a 10-year-old child with an odontoma within a dentigerous cyst (DC). The patient presented with a 1-week history of painful swelling involving the right mandible and cheek. An intraoral examination revealed a defect of the oral mucosa behind the permanent mandibular first molar, which resembled an exposed alveolar bone or tooth remnants. Clinical and radiographic evaluation revealed a large erupting OD associated with a dentigerous cyst. The OD was separated into pieces and enucleated together with the dentigerous cyst to preserve the mandible's integrity and because of the patient's age. This is the first report of an erupting OD associated with a DC in a child. Surgical removal might be a challenge when large ODs are encountered.

Morphometry of tumor cells in different grades and types of breast cancer.

The aim of the study was to compare morphometric characteristics of different types and grades of breast cancer. Morphometric analysis was performed using the SFORM software (Vamstec, Zagreb) on the May-Grünwald-Giemsa stained fine needle aspiration cytology (FNAC) breast tissue specimens. The study included 42 patients diagnosed with breast carcinoma by breast smear FNAC at Merkur University Hospital during the 2001-2005 period. Postoperative tumor histopathology and semi-quantitative tumor grading by the method of Elston and Ellis' showed invasive ductal carcinoma grade I in 10, invasive ductal carcinoma grade II in 9, invasive ductal carcinoma grade III in 13, and invasive lobular carcinoma in 13 patients, the latter also including a subtype of invasive tubulolobular carcinoma. The following parameters were assessed by use of Statistica 7.1 and chi2-test: tumor area, circumference, maximal radius, minimal radius, convexity, length, width, elongation, nucleus/cytoplasm ratio, and shape factor. Morphometric analysis yielded statistically significant differences among all study groups (p < 0.001). Morphometric parameters showed significant individual correlation with tumor type and grade, whereby the area, convexity and circumference were most significant at both nuclear and cellular level.

Mucoepidermoid carcinoma misdiagnosed as palatal odontogenic infection: an overview on the differential diagnosis of palatal lesions.

Mucoepidermoid carcinoma (MEC) accounts for approximately 30% of malignant salivary gland tumors and approximately 30% occur in minor salivary glands. The palate is the most frequent localization for those arising in minor glands. A 33-year-old male patient with MEC of the hard palate was treated as an acute odontogenic infection, which was not cured after tooth endodontic treatments, repeated incisions and antibiotics. On the hard palate ovoid, a hard painless mass, which had not extended over the middle palatal line, was observed. Partial maxillectomy was performed. A review of the literature was performed in order to provide a coherent overview on the differential diagnosis of palatal lesions. To the best of authors' knowledge, this is the first report in English literature describing palatal MEC misdiagnosed and treated as odontogenic infection. Considering the extensive list of MEC's differential diagnoses on the hard palate, acute odontogenic infection can now be added to that list.

Multidisciplinary Approach to Rehabilitation after Tumor Resective Jaw Surgery: A 9-Year Follow-Up.

A 36-year-old male patient presented at the Department of Maxillofacial Surgery, University Hospital Clinic Zagreb in December 2010 due to a swelling of the left body of the mandible that was noticed 4 months earlier. The patient was referred regarding an atypical clinical and radiological finding in the form of a multicystic appearance in the corpus of the left mandible and teeth mobility in the same region. A probatory biopsy was performed under local anesthesia and sent for histopathologic examination which reported odontogenic myxoma. The tumor was removed with a marginal resection of the mandible from the left first incisor to the left second molar. Two years after surgery, in January 2013, the patient was referred to the oral surgeons for implant-prosthodontic rehabilitation. Two narrow implants were placed at positions 32 and 36, and three months afterwards, implant-retained denture was made using locator connections to compensate lost teeth and to correct the ratio of soft tissues and facial contours. The patient was followed-up for 9 years without any functional and aesthetic problems. Loosing function and aesthetic morbidity, after radical surgical treatment, often have compromised the quality of life of this group of patients. It is important to highlight the need for multidisciplinary collaboration for the complete rehabilitation of the patient after surgical oncology of the maxillofacial region.

Pulmonary valve papillary fibroelastoma diagnosed by echocardiography: a case report.

Papillary fibroelastomas (PFEs) are rare cardiac valve tumours with reported incidence of <0.03% according to autopsy studies. Among them, pulmonary valve PFEs are extremely rare. With wider use of echocardiography, they are being increasingly recognized premortem. We describe a case of a 32-year-old Caucasian woman with PFE of pulmonic valve diagnosed by echocardiography. The patient underwent surgery due to high mobility of the tumour and high risk of embolic complications. The surgery was done with complete tumour resection and total preservation of valve function. This case report discusses diagnostics of PFEs, their characteristic echocardiographic and histological features, and possible complications and suggests treatment options in this rare cardiac tumour.

Case of Unrecognised of Maxillary Adenoid Cystic Carcinoma.

Adenoid cystic carcinoma is a less commonly diagnosed cancer that may affect the major or minor salivary glands. We present a 70 year old male patient who was admitted to the Department of Oral Medicine, School of Dental Medicine in Zagreb, Croatia due to pain in the right maxilla. In this case we report a case of the patient with unilateral pain in the maxilla & eye which lead to the diagnosis of adenoid cystic cancer without any visible oral lesions.

Genome-wide miRNA profiling reinforces the importance of miR-9 in human papillomavirus associated oral and oropharyngeal head and neck cancer.

Head and neck cancer is the sixth most common malignancy worldwide, predominantly developing from squamous cell epithelia (HNSCC). The main HNSCC risk factors are tobacco, excessive alcohol use, and the presence of human papillomavirus (HPV). HPV positive (+) cancers are etiologically different from other HNSCC and often show better prognosis. The current knowledge regarding HNSCC miRNA profiles is still incomplete especially in the context of HPV+ cancer. Thus, we analyzed 61 freshly collected primary oral (OSCC) and oropharyngeal (OPSCC) SCC samples. HPV DNA and RNA was found in 21% cases. The Illumina whole-genome small-RNA profiling by next-generation sequencing was done on 22 samples and revealed 7 specific miRNAs to HPV+ OSCC, 77 to HPV+ OPSCC, and additional 3 shared with both; 51 miRNAs were specific to HPV- OPSCC, 62 to HPV- OSCC, and 31 shared with both. The results for 9 miRNAs (miR-9, -21, -29a, -100, -106b, -143 and -145) were assessed by reverse transcription-quantitative polymerase chain reaction on the whole study population. The data was additionally confirmed by reanalyzing publicly available miRNA sequencing Cancer Genome Atlas consortium (TCGA) HNSCC data. Cell signaling pathway analysis revealed differences between HPV+ and HPV- HNSCC. Our findings compared with literature data revealed extensive heterogeneity of miRNA deregulation with only several miRNAs consistently affected, and miR-9 being the most likely HPV related miRNA.

Prognostic significance of bone morphogenetic protein 6 (BMP6) expression, clinical and pathological factors in clinically node-negative oral squamous cell carcinoma (OSCC).

Bone morphogenetic protein 6 (BMP6) has unique properties regarding structure and function in supporting bone formation during development and adult life. Despite its known role in various malignant tumors, the prognostic significance of BMP6 expression in oral squamous cell carcinoma (OSCC) remains unknown. The aim of the study was to investigate immunohistochemical expression of BMP6 in OSCC in correlation with clinical and pathological parameters, disease recurrence and survival. In addition, we investigated other parameters in order to identify prognosticators of neck metastases and final outcome. The study included 120 patients with clinically T1-3N0 OSCC who were primarily surgically treated between 2003 and 2008. There were 99 (82.5%) male and 21 (17.5%) female patients. The five-year disease-specific survival for the whole cohort was 79.7%. Tumors smaller than 2 cm in diameter showed higher incidence of strong BMP6 expression. No statistical correlation was observed between other clinico-pathological factors and BMP6 expression. Expression of BMP6 was not associated with disease recurrence and survival. BMP6 may not serve as prognosticator of final outcome or recurrence in clinically node-negative OSCC subjects. In multivariate analysis predictors of poorer survival were positive surgical margin, moderate tumor cell differentiation and pathological involvement of levels IV and/or V.