Potential For Aggressive Behavior Research Articles

Basaloid Squamous Cell Carcinomas of the Thymus With Prominent B-Cell Lymphoid Hyperplasia: A Clinicopathologic and Immunohistochemical Study of 10 Cases.

Ten cases of basaloid squamous cell carcinomas of the thymus are presented. The patients are 6 women and 4 men ranging in ages between 51 and 72 years (average: 61.5y), who presented with nonspecific symptoms of cough, dyspnea, and chest pain with no history of malignancy, myasthenia gravis, or other autoimmune disease. Surgical resection of the mediastinal masses via thoracotomy or sternotomy was performed in all patients. Grossly, the tumors varied in size from 2 to 8cm, were light tan in color, solid and slightly hemorrhagic, and had infiltrative borders. Histologically, scanning magnification showed elongated interanastomosing ribbons of tumors cells embedded in a lymphoid stroma containing germinal centers. At higher magnification, the tumors cells were round to oval with moderate amounts of lightly eosinophilic cytoplasm, oval nuclei, moderate cellular atypia, and mitotic activity ranging from 3 to 5 mitotic figures per 10HPFs. In 8 cases, the tumor invaded perithymic adipose tissue, in 1 case the tumor infiltrated pericardium, and in 1 case, the tumor involved the pleura. Immunohistochemical stains showed positive staining in the epithelial component for pancytokeratin, p63, keratin 5/6, and p40, while CD20 and CD79a characterized the lymphoid component. Clinical follow-up was obtained in 7 patients. Two patients died within 24 months and 5 patients remained alive between 12 and 60 months. The current cases highlight the unusual feature of B-cell lymphoid hyperplasia in these tumors and their potential aggressive behavior.

Extranodal Follicular Dendritic Cell Sarcoma of the Head and Neck Region: A Clinicopathological Study of 7 Cases.

Background. Follicular dendritic cell (FDC) sarcoma is a rare neoplasm arising from follicular dendritic cells (FDCs). It can be nodal or extranodal. Histological diagnosis of extranodal FDC sarcoma in the head and neck region is challenging and a significant percentage are misdiagnosed. Objectives. To report clinicopathological features of head and neck extranodal FDC sarcoma cases and discuss differential diagnoses. Methods. Seven head and neck extranodal FDC sarcomas were retrieved and clinicopathological features were noted. Results. Two tumors each involved parapharyngeal space and tonsil while remaining cases involved the parotid, soft tissue of neck and oropharynx. Age range was 12 to 79 years (mean and median age were 40 and 44 years respectively) and there was a male predilection (6 males: 1 female). All showed spindle to ovoid cells arranged in fascicles, whorls and/or storiform pattern. Mitoses ranged from 3 to 20/mm2. All tumors expressed CD21 and CD23. Two patients died of their disease at 9 and 16 months. Both had tumors larger than 5 cm with ≥10 mitoses/mm2. Three patients were alive at 12, 44 and 184 months. Conclusions. There was a distinct male predominance in our cohort. FDC sarcoma should be included in the differential diagnosis of spindle cell extranodal neoplasms in the head and neck with a whorled growth pattern and intratumoral lymphocytes. Head and neck region tumors show similar clinicopathologic characteristics as their counterparts at other locations with potential for aggressive behavior especially in tumors greater than 5 cm in size and with high mitotic rates.

Expanding the spectrum of mesenchymal neoplasms with NR1D1-rearrangement.

Undifferentiated mesenchymal neoplasms can be morphologically subclassified based on cell shape; epithelioid tumors may be diagnostically challenging, particularly since they can show morphologic and immunohistochemical overlap with epithelial neoplasms. Following the recent report of an NR1D1::MAML1 gene fusion in an undifferentiated pediatric neoplasm, we performed a retrospective archival review and identified four additional cases of undifferentiated mesenchymal neoplasms with NR1D1-rearrangement. All four tumors occurred in adult women. The tumors involved superficial and/or deep soft tissues of the extremities or abdomen. Morphologically, they showed a spectrum of overlapping features. In addition to epithelioid cells, two cases also had a prominent spindle cell component. Two cases also had admixed polygonal cells containing prominent cytoplasmic vacuoles with amorphous debris. The immunophenotype was nonspecific but all cases had at least focal keratin expression; this was extensive in two tumors. Targeted RNA-sequencing revealed two cases each with NR1D1::MAML1 and NR1D1::MAML2 gene fusions. One patient developed lung and liver metastases, and one patient required amputation due to multifocal disease and underlying bone involvement. This study confirms undifferentiated NR1D1-rearranged sarcoma represents a distinct mesenchymal neoplasm with an epithelioid morphology and potential for aggressive behavior. Further, we offer new insight into the spectrum of clinical, morphologic, immunohistochemical, and molecular findings possible in these rare neoplasms. An awareness of this entity is especially important given the potential for misclassification as a carcinoma.

Particularities of the treatment of odontogenic keratocyst by cystectomy. Clinical case

Odontogenic keratocysts (KO) are benign uni- or multichystic, intraosseous lesions of development located in the oro-maxillo-facial region. They appear as a result of the disruption of odontogenesis, the main sources being the dental lamina with its remains and the extensions of the basal layer of the oral covering epithelium. It is characterized by a potential aggressive, infiltrative or diffuse behavior. Due to the fact that the condition is associated with an asymptomatic clinical picture, it is diagnosed late, when the formation is in a phase of chronic evolution, reaching considerable dimensions. Thus, there is a dilemma among specialists regarding the choice of the optimal treatment method, addressing the need to combine surgical-conservative methods with surgical methods that have a radical character, such as mandibular resection. The aim of the study is the early diagnosis of odontogenic keratocyst and the treatment of the disease in a minimally invasive way. The study examined a patient who was diagnosed with odontogenic keratocyst and treated by the cystectomy method. Analyzing the data of the literature and the aspects of the treatment of odontogenic keratocysts, it was found that the surgical management of patients with KO is individually being influenced by the high recurrence rate and its morphopathological peculiarities.

Trichoblastic carcinoma: a comprehensive review of the literature.

Trichoblastic carcinoma is a rare neoplasm thought to arise from malignant degeneration of benign follicular tumors. Its clinical features, optimal treatment, and outcomes remain largely unknown. We performed a comprehensive review of the existing trichoblastic carcinoma literature. PubMed and Google Scholar were searched for keywords related to trichoblastic carcinoma, and additional articles were found in reference lists. Cases with a histopathologic diagnosis of trichoblastic carcinoma were summarized. A total of 93 cases have been described, all in case reports or case series. The average age was 65, with 66.7% of cases in males. A minority of patients had familial tumor syndromes or a history of radiation at the site, and only one was reported to be immunosuppressed. The most common site was the face (48.4%), and the majority developed de novo (87.1%). The clinical presentation was highly variable. Although most cases (82.8%) were successfully treated with surgery alone, a subset of patients had more aggressive disease including local progression or recurrence in five cases, nodal metastases in five cases, and distant metastases in three cases. Trichoblastic carcinoma is a rare malignancy with the potential for aggressive behavior. Further research is needed to better characterize this neoplasm.

Correlation between Parenting Styles and Peer Attachment with Aggressive Behavior Potentials in Adolescent Boys

Background: Adolescence is a transitional phase that causes biological, cognitive, social-emotional, and behavioral changes. One of the issues of concern is aggressive behavior in adolescents. Parenting styles and peer attachment are one of the factors that have an influence on potential aggressive behavior in adolescent boys. Objectives To analyze the relationship between parenting styles and peer attachment to the potential aggressive behavior of adolescent boys. Method: The subjects were junior high school male students who are cared for by both biological parents and can follow all of the research procedures. The research design was cross-sectional with total sampling technique. Subjects performed anamnesis related data self, parent demographic data, and peers. (PAQ), (IPPA) (BPAQ). The analyzes used were Pearson, Spearman, and Anova. Results: The majority of parenting styles was permissive (34.06%; 48.00%). Parenting styles and peer attachments did not have a significant relationship with potential aggressive behavior (p = 0.798; p = 0.071; p = 0.695). There was a negative correlation of peer attachment with alienation dimension (p = 0.001). Other significant dimensions with potential dimensions of aggressive behavior were trust (p = 0.018; 0.022) and communication (p = 0.029; p = 0.000). Conclusion: Parenting styles and peer attachments did not have a meaningful relationship with potential aggressive behavior in adolescent boys. However, there were some significant correlations between the dimensions of parenting and peer attachment with potential dimensions of aggressive behavior.

Intraosseous Melanotic Schwannoma in the Sacrum Mimicking Primary Bone Tumor

Primary tumors of sacrum are rarely seen, and the differential diagnosis is extensive, such as chordomas, giant cell tumors, and schwannomas. Sacral intraosseous schwannomas (IOSs) are very rare and encompass approximately 1%–5% of all spinal schwannomas. Melanotic schwannomas (MSs) are categorized as an unusual variant of benign schwannomas; however, they sometimes follow a malignant course. The authors present a case of MS with intraosseous extension into sacrum in a 48-year-old male arising from the left S2 nerve root. Magnetic resonance imaging (MRI) and computed tomography (CT) scan demonstrated a destructive mass in the sacrum. He was made a diagnosis with MS by 18F-fluoro-deoxy-glucose positron-emission-tomography (18F-FDG PET) and open biopsy. The tumor was blackish-colored and vascular-rich fragile tumor covered by fibrous capsule. The floor of the tumor was not encapsulated and invading into the sacral bone. Total removal of the tumor together with the left S2 nerve of origin via posterior approach was achieved. The patient made dramatic recovery of neurological symptoms and tumor recurrence is not seen for 6-month follow-up period. MS is a benign tumor with potential for aggressive behavior and capacity to metastasize. Therefore, total removal of the tumor and careful postoperative follow-up are recommended. Postoperative spinopelvic stability also needs to be taken into consideration. The authors discuss our successful management with a focus on diagnostic process, surgical planning, and histological consideration to provide the most up-to-date guidance on managing this challenging tumor.

Neutrophil: Lymphocyte ratio and oral squamous cell carcinoma: A preliminary study.

Introduction/Background:It is well-established that host response plays a vital role in the determination of biological behavior of tumors. Inflammatory response against tumors has been a focus of research in recent years. There has been extensive research regarding inflammatory reaction in oral squamous cell carcinoma (OSCC) in tissue specimens. However, there is no much data regarding systemic inflammatory response in OSCC. It was recently reported that an elevated neutrophil:lymphocyte ratio (NLR) may correlate with aggressive biological behavior in various malignancies including head-and-neck tumors. Thus, the study was undertaken with the aim of evaluation of NLR in peripheral blood of patients suffering from OSCC.Materials and Methods:The research was a prospective, case–control study. Hundred patients suffering from OSCC and 100 healthy individuals were included in the study, after obtaining informed consent. NLR was determined from the differential leukocyte count obtained from complete blood count in each case. Comparison of NLR was made among two groups.Results:The mean value of absolute neutrophil count was found to be significantly higher in OSCC cases than in controls (P < 0.01). The mean value of NLR in OSCC was found to be 2.84, and in controls, it was 1.95. This difference was found to be statistically significant (P < 0.001).Conclusion:NLR and other hematological parameters may serve as surrogate marker for potential aggressive behavior of OSCC and may help in prognostic prediction of these cases.

Pulmonary Carcinomas With Mucinous and Neuroendocrine Differentiation: Expanding the Spectrum of Amphicrine Carcinomas.

Amphicrine carcinoma is a distinct type of carcinoma characterized by synchronous exocrine and endocrine differentiation within the same tumor cell. Such tumors are exceedingly rare and most commonly recognized in the gastrointestinal tract. In the lung, sporadic descriptions of such lesions exist. This report presents 3 more such tumors, expanding the spectrum of amphicrine carcinomas in this organ. The patients were 3 men, 53 to 78 years of age (mean: 63 y) and all were current or former smokers. In one patient the tumor was an incidental finding, the others presented with lower respiratory symptoms. Microscopically, the lesions had an organoid pattern, consisting of nests of large tumor cells with peripheral palisading, vesicular nuclei and prominent nucleoli reminiscent of large cell neuroendocrine carcinoma. In addition, the tumor nests also showed glandular features, characterized by gland-like lumina filled with mucin and intracytoplasmic mucin droplets. Immunohistochemical studies showed diffuse reactivity of the tumor cells with synaptophysin and chromogranin and variable reactivity with TTF-1. Mucin stains highlighted extracellular and intracellular mucin deposition. Clinical follow-up demonstrated that one patient had died of his disease 12 months after surgery while another was alive and free of disease 21 months after surgery. The third patient was alive with widespread metastatic disease 8 months after diagnosis. This series highlights a rare variant of pulmonary carcinoma showing synchronous exocrine and endocrine differentiation. Recognition of this type of tumor is important in terms of tumor classification and to identify an entity with potential aggressive behavior.

Pulmonary and mediastinal paragangliomas: rare endothoracic malignancies with challenging diagnosis and treatment

Pulmonary and mediastinal paragangliomas are rare tumors that may have neuroendocrine activity or be non-functional, incidental, in asymptomatic patients, or causing mass effect symptoms. Although being low-grade tumors, they can display an aggressive behaviour, developing local infiltration and distant metastases. We report our experience with three endothoracic paragangliomas and a Literature review, to point out diagnostic difficulties and problems related to surgical treatment. From 2009 to 2017, we treated 3 patients with histological diagnosis of paraganglioma: 2 pulmonary, 1 mediastinal. No one presented catecholamine-secreting syndromes; pulmonary cases were asymptomatic, while the mediastinal one had aspecific cough and dyspnea. Imaging diagnosis was based on chest computerized tomography (CT) and magnetic resonance imaging (MRI) scan. No patient had preoperative histological diagnosis. Intraoperative pathological examination was suggestive for malignancy: in pulmonary cases, wedge resection and lobectomy were performed; the middle mediastinal mass was completely removed after challenging dissection, isolation and section of numerous vascular pedicles. Postoperative course was uneventful in all cases. No patient received adjuvant treatments. At a median follow-up of 47 months (range, 6-102 months), two patients are alive, without local or distant recurrence; one patient died 6 months after surgery, due to disease progression. Endothoracic paragangliomas, rare and often asymptomatic tumors, are of difficult diagnosis and should be considered malignant tumors, due to the potential aggressive behaviour of cases with high mitotic index and the frequent possibility of recurrence and metastases. Surgical resection is the treatment of choice and careful intraoperative manipulation is recommended, due to the high vascularity of these tumors, to prevent complications. After complete excision, long-term prognosis is generally good. However, even after surgical removal, a close, periodical and life-long follow-up is mandatory.

CALCITONIN SECRETORY INDEX AND UNSUSPECTED NODAL DISEASE IN MEDULLARY THYROID CARCINOMA

Medullary thyroid carcinoma (MTC) is a rare thyroid malignancy originating from parafollicular C-cells with the potential for aggressive behavior. The extent of lymph node (LN) dissection at the time of surgery is controversial, with different schools of thought prevailing. Some systematically perform LN dissections, whereas others base their decision on radiologic evidence of disease and some with the assistance of pre-operative calcitonin (CT) levels. We retrospectively assessed the correlation between pre-operative CT levels and clinico-pathologic factors among 42 patients with MTC between 1994 and 2015. Furthermore, we refined the use of pre-operative serum CT levels and explored for the first time a test called the Calcitonin Secretory Index (CSI, ng/mL/mm). Pre-operative CT levels correlated independently with tumor size ( P<.0001), number of metastatic LNs ( P<.01), and increased rates of distant metastasis. The CSI better predicted early LN disease ( P<.045). Patients with early LN metastasis had a CSI >30 ng/mL/mm, a representative threshold above which the surgical cure declines considerably. In our experience, pre-operative CT levels and now the CSI appear as sensitive and specific risk stratification markers for MTC. Despite negative findings on dedicated pre-operative neck imaging in addition to total thyroidectomy, a CSI >30 ng/mL/mm would prompt bilateral central node dissection. Due to the small sample size, our study provides preliminary evidence of the value of CSI in clinical practice. ANOVA = analysis of variance; ATA = American Thyroid Association; CSI = Calcitonin Secretory Index; CT = calcitonin; LN = lymph node; MTC = medullary thyroid carcinoma; ROC = receiver operating characteristic.

P2.17-002 Pulmonary and Mediastinal Paragangliomas: Rare Endothoracic Malignancies with Challenging Diagnosis and Treatment

Tumors originating from chromaffin cells are located in 90% of cases in the adrenal gland and called pheochromocytomas, while in 10% of cases have an extra-adrenal origin (paraganglionic cells scattered throughout the body) and are termed paragangliomas. Endothoracic paragangliomas can arise in the lung and mediastinum, may have neuroendocrine activity, secreting catecholamines, or be non-functional, incidental, in asymptomatic patients or causing mass effect symptoms. Even if rare and with low grade of malignancy, they can present an aggressive behavior, developing local infiltration of surrounding organs and distant metastases. A correct pathological diagnosis and radical surgical treatment are fundamental to obtain clinical recovery. We report our experience with three cases of endothoracic paragangliomas, in order to point out difficulties in diagnosis and problems related to surgical treatment. From January 2009 to December 2016, we treated 3 patients (2 women, 1 man), mean age 67 years, with histological diagnosis of paraganglioma: 2 pulmonary, 1 mediastinal. All patients were asymptomatic for catecholamine-secreting syndromes; the two cases with pulmonary localization showed no other symptoms, while the mediastinal one had cough and dyspnea, due to compression by the mass, and blepharospasm. Imaging diagnosis was based on chest CT scan in pulmonary cases, chest CT and MRI scan for the mediastinal mass, which allowed to define vascularization and relationships with adjacent structures. No patient had preoperative histological diagnosis. Intraoperative pathological examination of the two pulmonary forms was suggestive for malignancy (extensive necrosis, high proliferative index, hypervascularization), thus a right pulmonary wedge resection was performed in one case, a right upper lobectomy in the other one. The hypervascularized mediastinal lesion, located in the middle mediastinum, tenaciously adherent to the superior vena cava, the aortic arch, the right branch of the pulmonary artery and the trachea, was completely removed after challenging isolation and section of numerous vascular pedicles. Postoperative course was uneventful in all cases. No patient received adjuvant treatments. At a median follow-up of 35 months (range: 6-90 months), two patients are alive, without local or distant recurrence; one patient (with pulmonary involvement) died 6 months after surgery, due to disease progression. Endothoracic paragangliomas, rare and often asymptomatic tumors, are of difficult diagnosis and should be considered malignant tumors, due to the potential aggressive behavior of cases with high mitotic index and the frequent possibility of recurrence. After complete excision, long-term prognosis is generally good. However, even after surgical removal, a close and periodical follow-up is mandatory.

Proliferaton index in pituitary adenomas from a black African population

BackgroundThe WHO has recognized a variant of pituitary adenomas with potential aggressive behaviour which have been termed atypical pituitary adenomas. This group of tumours are recognized by their mitotic rate of more than >3%, p53 expression and invasion of surrounding structures. There has however been no study of the occurrence of these tumours in a black African population. This study is a preliminary attempt to examine this group of tumours in blacks. MethodsThis study retrospectively reviewed fifty-seven histologically diagnosed and immunohistochemically characterized pituitary adenomas received in our department over a twenty-one year period. Specimens were stained with ki67, a nuclear marker of cell proliferation which has been identified as the single best predictor of atypical pituitary adenoma. ResultsTwelve of the tumours showed atypical features with eight (67%) of these tumours being prolactinomas. Two of the tumours were gonadotrophs and two were null cell adenomas. There was no correlation with age or gender. Two of the tumours required neurosurgical re-exploration with one of these showing a higher mitotic index in the second biopsy. ConclusionThe study suggests similarity in the rate of occurrence of pituitary adenomas with atypical features in a black African population with what is seen in Caucasians. Prolactinomas constitute a significant percentage of the tumours with this feature.

Genomic profiling of malignant phyllodes tumors reveals aberrations in FGFR1 and PI-3 kinase/RAS signaling pathways and provides insights into intratumoral heterogeneity

AbstractMalignant phyllodes tumors of the breast are poorly understood rare neoplasms with potential for aggressive behavior. Few efficacious treatment options exist for progressed or metastatic disease. The molecular features of malignant phyllodes tumors are poorly defined, and a deeper understanding of the genetics of these tumors may shed light on pathogenesis and progression and potentially identify novel treatment approaches. We sequenced 510 cancer-related genes in 10 malignant phyllodes tumors, including 5 tumors with liposarcomatous differentiation and 1 with myxoid chondrosarcoma-like differentiation. Intratumoral heterogeneity was assessed by sequencing two separate areas in 7 tumors, including non-heterologous and heterologous components of tumors with heterologous differentiation. Activating hotspot mutations in FGFR1 were identified in 2 tumors. Additional recurrently mutated genes included TERT promoter (6/10), TP53 (4/10), PIK3CA (3/10), MED12 (3/10), SETD2 (2/10) and KMT2D (2/10). Together, genomic aberrations in FGFR/EGFR PI-3 kinase and RAS pathways were identified in 8 (80%) tumors and included mutually exclusive and potentially actionable activating FGFR1, PIK3CA and BRAF V600E mutations, inactivating TSC2 mutation, EGFR amplification and PTEN loss. Seven (70%) malignant phyllodes tumors harbored TERT aberrations (six promoter mutations, one amplification). For comparison, TERT promoter mutations were identified by Sanger sequencing in 33% borderline (n=12) and no (0%, n=8) benign phyllodes tumors (P=0.391 and P=0.013 vs malignant tumors, respectively). Genetic features specific to liposarcoma, including CDK4/MDM2 amplification, were not identified. Copy number analysis revealed intratumoral heterogeneity and evidence for divergent tumor evolution in malignant phyllodes tumors with and without heterologous differentiation. Tumors with liposarcomatous differentiation revealed more chromosomal aberrations in non-heterologous components compared with liposarcomatous components. EGFR amplification was heterogeneous and present only in the non-heterologous component of one tumor with liposarcomatous differentiation. The results identify novel pathways involved in the pathogenesis of malignant phyllodes tumors, which significantly increase our understanding of tumor biology and have potential clinical impact.

Giant Cell Tumor of Bone - An Overview.

Giant Cell tumors (GCT) are benign tumors with potential for aggressive behavior and capacity to metastasize. Although rarely lethal, benign bone tumors may be associated with a substantial disturbance of the local bony architecture that can be particularly troublesome in peri-articular locations. Its histogenesis remains unclear. It is characterized by a proliferation of mononuclear stromal cells and the presence of many multi- nucleated giant cells with homogenous distribution. There is no widely held consensus regarding the ideal treatment method selection. There are advocates of varying surgical techniques ranging from intra-lesional curettage to wide resection. As most giant cell tumors are benign and are located near a joint in young adults, several authors favor an intralesional approach that preserves anatomy of bone in lieu of resection. Although GCT is classified as a benign lesion, few patients develop progressive lung metastases with poor outcomes. Treatment is mainly surgical. Options of chemotherapy and radiotherapy are reserved for selected cases. Recent advances in the understanding of pathogenesis are essential to develop new treatments for this locally destructive primary bone tumor.

Equine Cutaneous Mast Cell Tumours Exhibit Variable Differentiation, Proliferation Activity and KIT Expression

Equine cutaneous mast cell tumours (CMCTs) are generally considered to be benign skin lesions, although recurrent and multicentric tumours have been described. For canine CMCTs, grading and prognostic approaches are well established and aberrant KIT expression as well as high proliferation indices are associated with poor outcome. However, in the case of equine CMCTs, morphological features, proliferative activity and KIT expression pattern have not been assessed or related to biological behaviour, and there is discussion as to whether CMCTs are true neoplastic processes. The present study describes 45 equine CMCTs in terms of their morphology and KIT and PCNA expression by immunohistochemistry. KIT expression was classified as membranous (I), cytoplasmic and focally stippled (II) or diffuse cytoplasmic (III). A large proportion of the tumours were multinodular or diffuse dermal infiltrates of mast cells with mild anisokaryosis, a low proliferative rate and a dominance of KIT pattern I, representing well-differentiated CMCTs. In approximately one third of the cases, the mast cells exhibited more infiltrative growth, moderate to marked anisokaryosis and a higher degree of proliferation. These were classified as poorly differentiated CMCTs and exhibited only KIT patterns II and III. These findings indicate that there is a subgroup of poorly differentiated equine CMCTs, in which there is an association between aberrant KIT expression, high proliferative rate and potential aggressive behaviour, all features that confirm at least the poorly differentiated CMCT as a true neoplastic processes.

Morphologically low-grade spiradenocarcinoma: a clinicopathologic study of 19 cases with emphasis on outcome and MYB expression

Spiradenocarcinoma is a rare skin adnexal neoplasm with potential for aggressive behavior, classified histologically into low- and high-grade tumors. Morphologically, low-grade tumors are thought to behave more favorably. Limited information is available, however, with only 18 published cases. To study their clinical behavior, histological features, and the diagnostic value of immunohistochemistry, 19 morphologically low-grade spiradenocarcinomas were retrieved and compared with 21 spiradenomas and cylindromas. H&E-stained sections were reviewed, follow-up was obtained, and immunohistochemistry for Ki-67, p53 and, MYB was performed. The tumors were solitary, measuring 0.8–7 cm (median: 2.7 cm), with a predilection for the head and neck of elderly patients (median age: 72 years; range 53–92) without gender bias. Histologically, the tumors were multinodular and located in deep dermis and subcutis. A pre-existing spiradenoma was present in all cases. The malignant component was characterized by expansile growth with loss of the dual cell population, up to moderate cytological atypia and increased mitotic activity (median: 10/10 HPF; range 1–28). Additional findings included squamoid differentiation (n=9), necrosis (n=7), and ulceration (n=5). P53 expression was variable and no significant differences were noted in the benign compared with the malignant parts of the tumors. In contrast, in the malignant components the Ki-67 proliferative index was slightly increased, and MYB expression was lost. Follow-up (median: 67 months; range: 13–132) available for 16 patients (84%) revealed a local recurrence rate of 19% but no metastases or disease-related mortality. In this large study with long-term follow-up, we demonstrate that spiradenocarcinomas with low-grade morphology pursue an indolent course, characterized by local recurrence only. Metastases and disease-related mortality appear to be exceptional. Lack of MYB expression may be useful as an additional aid in the diagnosis of these challenging tumors.

Hyalinizing clear cell carcinoma of the head and neck: Case series and update.

Hyalinizing clear cell carcinoma (HCCC) is a rare neoplasm of salivary gland origin which is low-grade in nature. In this series, we report on 9 cases of HCCC and update the literature. A comprehensive literature search was performed. Our institutional database was queried for cases of HCCC. Pathology reports and slides were reviewed. Data was extracted, including demographics, staging, primary treatment, histopathologic findings, recurrence, and disease status. Statistical analysis was performed using a Pearson's chi-square test. One hundred thirty-six total cases were included for analysis. The overall recurrence rate was 19.8%. Necrosis was associated with risk of recurrence (p = .003). Positive margins and lymph node status were associated with risk of recurrence. Prognosis was good with only 3 of 82 patients reported dying with disease. HCCC has an overall good prognosis with potential for aggressive behavior. The presence of necrosis, locoregional disease, or positive margins is associated with risk for recurrence.

Synovial chondromatosis of the tempromandibular joint: Relationship between MRI information and potential aggressive behavior

PurposeTo evaluate the types of synovial condromatosis (SC) of tempromandibular joint (TMJ) on MRI and their potential aggressive behavior. Methods144 patients with pure SC of TMJ were included in. On MRI, 3 types of the lesion included loose body, homogeneous mass, and mixture of both loose body and homogeneous mass. Bony evaluations included 4 stages of erosion: without erosion, chondral breakdown, bony absorption and bony perforation. Results47 (32.6%) cases were categorized in the type of loose body, 71 (49.3%) cases in the type of homogeneous mass, and 26 (18.1%) cases in the type of mixture. 80% of bony perforation belonged to the type of homogeneous mass and 20% belonged to the type of mixture. 2 patients were found recurrence. ConclusionThe types of homogeneous mass and mixture of both on MRI were more aggressive. Homogeneous mass with bony erosion on MRI had potential recurrence.

기저세포모반증후군 : 증례 보고

기저세포모반증후군은 상염색체 우성의 유전 질환이며 주요소견으로는 다발성 기저세포암, 악골의 다발성 각화낭종, 늑골이상, 손과 발바닥의 소와, 대뇌겸의 석회화 등이 있다. 기저세포모반증후군에서 호발 하는 각화낭성 치성종양은 치성기원의 골내종양으로 착각화된 중층 편평상피에 의해 특징적으로 이장되어 있고 공격적이고 침습적인 성향을 갖는 것으로 정의된다. 본 증례는 감별진단이 필요한 상악 견치부위의 단방성의 낭종을 주소로 내원한 환아가 조직검사를 통해 각화낭성 치성종양으로 밝혀진 후 임상적, 방사선학적 검사를 통해 기저세포모반 증후군으로 진단된 경우이다. 종양은 적출술로 치료되었고 증후군과 연관된 추가적인 질환들의 조기진단을 위해 정기적인 검진이 필요할 것이다. Nevoid basal cell carcinoma syndrome(NBCCS) is a autosomal dominant disorder, and its major manifestations are multiple basal cell carcinoma, keratocystic odontogenic tumor, rib anomalies, palmer and plantar pits, calcification of the falx cerebri. Keratocystic odontogenic tumor(KCOT) is defined as intraosseous tumor of odontogenic origin with a characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive behavior. We report a case of a 3-year-old patient with nevoid basal cell carcinoma syndrome who initially presented with unilocular keratocystic odontogenic tumor in maxillary canine region. Keratocystic odontogenic tumor was treated by enucleation, and periodic follow-up check will be required for early diagnosis of additional diseases related with this syndrome.