Nasal Eosinophilic Angiocentric Fibrosis with Orbital Extension
2015-09-01 03:00:00 AM
Abstract
Eosinophilic angiocentric fibrosis (EAF) is an extremely rare, chronic, benign, idiopathic disorder that mostly affects the upper respiratory tract, particularly the nasal cavity, and features progressive submucosal perivascular fibrosis. To the best of our knowledge, only seven cases of EAF with orbital involvement have been reported. We report a case of sinonasal EAF with orbital extension that presented with left nasolacrimal duct obstruction. A 35-year-old man presented with left epiphora, proptosis, anterolateral globe displacement and nasal obstruction. Endoscopic sinus examination showed a firm, gritty, creamy, yellow, fibrous, adherent mass of maxillary sinus. Diagnosis was established with histopathological examination of excisional biopsy of the lesion. Although EAF is very rare, it should be considered in the differential diagnosis of lesions of upper airway tract, particularly the nasal cavity. Biopsy is necessary for diagnosis and treatment planning. Resecting of the involved tissues completely is essential for prevention of recurrence.
Hodgkin Lymphoma of the Nasopharynx: Case Report with Review of the Literature
2015-09-01 03:00:00 AM
Abstract
We report an uncommon case and histopathologic work-up of Hodgkin lymphoma of the nasopharynx in a 49-year old female patient who presented with a 2-year complaint of bilateral nasal congestion. Histologic study revealed a lymphocyte rich subtype of classic Hodgkin lymphoma. Immunohistochemical analysis revealed CD15, CD30, OCT-2, BOB.1, and MUM-1 expression by the neoplastic cells and a lack of expression of CD45, CD20, CD3, EMA, and EBER. The review of the literature showed that Hodgkin lymphoma of the nasopharynx is rare, the most common reported subtype is the mixed cellularity, and Hodgkin lymphoma of the nasopharynx has a favorable prognosis.
Second Branchial Cleft Cyst
2015-09-01 03:00:00 AM
Dermoid Cyst of the Floor of the Mouth
2015-09-01 03:00:00 AM
Abstract
A case of a dermoid cyst of the floor of mouth affecting a 19 years old male will be discussed. The macroscopic and histologic findings used for diagnosis will be covered. The typical features of this type of cyst will also be reviewed including radiographic and histologic findings. Discussion will include etiology and usual presentation, as well as treatment.
Stensen's Duct Carcinoma with a Papillary Architecture
2015-09-01 03:00:00 AM
Abstract
Primary carcinoma of the parotid duct (Stensen's duct carcinoma) is a rare entity, first described in 1927 and with approximately thirty-one cases reported in the English literature. Criteria for diagnosis are primarily demonstration of an origin from the Stensen's duct lining and exclusion of parotid gland, accessory parotid, oral mucosal and adjacent minor salivary gland origin. The carcinoma is usually of a specific type, and most have been described as squamous, mucoepidermoid, or undifferentiated adenocarcinomas. We report an unusual case of Stensen's duct carcinoma showing a primarily basaloid phenotype with focal squamous differentiation and a partial papillary architecture raising the possibility of malignant transformation in a ductal papilloma. Wide local excision was performed with postoperative radiotherapy and the patient is free of complications one and a half years postoperatively. Due to the small number of cases reported, the overall prognosis is not well defined, but seems to depend on the tumour size. Regional metastasis confers a 14 % mortality rate but there appears to be no relationship between histological type and prognosis.
A Rare Case of Carcinoma Cuniculatum Involving the Larynx in Association with a Saccular Cyst
2015-09-01 03:00:00 AM
Abstract
Carcinoma cuniculatum (CC), a rare, well-differentiated variant of squamous cell carcinoma, is uncommon in head and neck sites but when it does occur is most common in the oral cavity. Here we report a rare case of CC involving the larynx. A 49-year-old man presented with 10 months of worsening hoarseness and, despite multiple biopsies, no diagnosis of malignancy could be established. Eventual partial excision of the lesion and histologic review of prior specimens confirmed the diagnosis of CC. Focally, a transition to respiratory epithelium indicated the presence of an associated saccular cyst. Total laryngectomy was performed and 6 months later the patient is free of disease. Only two prior cases of CC have been reported in the larynx. Diagnosis of CC is challenging given the low grade histologic features and awareness of this entity for both treating physicians and the pathologist is important to reach a diagnosis of malignancy. This case highlights the challenges in diagnosis of CC, especially in unusual locations and when associated with other lesions such as a saccular cyst. Awareness of this rare tumor type combined with close communication between treating clinicians, radiologists and pathologists should allow earlier diagnosis and treatment.
Report of Two Cases of Combined Odontogenic Tumors: Ameloblastoma with Odontogenic Keratocyst and Ameloblastic Fibroma with Calcifying Odontogenic Cyst
2015-09-01 03:00:00 AM
Abstract
Combined odontogenic neoplasms have rarely been documented. Such tumors have also been described by other researchers as "hybrid" lesions. The histologic features are often identical to other individually well-established odontogenic neoplasms such as ameloblastoma, adenomatoid odontogenic tumor, ameloblastic fibroma (AF), and ameloblastic fibro-odontoma. Their clinical presentation is variable, ranging from cysts to neoplasms showing varying degrees of aggressive behavior. Most combined tumors contain features of one of the odontogenic tumors in combination with either a calcifying odontogenic cyst (COC) or a calcifying epithelial odontogenic tumor. We present two new cases of combined odontogenic tumors: an ameloblastoma with an odontogenic keratocyst and an AF with COC. Predicting clinical outcome is challenging when a combination tumor is encountered due to the paucity of such lesions. One must understand salient features of these entities and differentiate them from the more common conventional neoplasms to expand classification and provide prognostic criteria.
Parotid Gland Nodular Fasciitis: A Clinicopathologic Series of 12 Cases with a Review of 18 Cases from the Literature
2015-09-01 03:00:00 AM
Abstract
Nodular fasciitis (NF), very uncommon in the parotid gland, is a benign myofibroblastic proliferation that may be mistaken for other neoplastic proliferations. The mass-like clinical presentation and histologic features result in frequent misclassification, resulting in inappropriate clinical management. There are only a few reported cases in the English literature. Cases within the files of the authors' institutions (retrospective) confined to the parotid gland were compared to cases reported in the English literature (Medline 1966–2014). The patients included five females and seven males, aged 11–70 years (mean 45.2 years). All patients presented with a mass lesion, present on average 1.9 months, without a documented history of trauma. The lesions were 0.7–5.2 cm (mean 2.2 cm). Seven patients had fine needle aspiration. The majority of the lesions were circumscribed (n = 9), composed of spindle-shaped to stellate myofibroblasts (MF) arranged in a storiform growth pattern, juxtaposed to hypocellular myxoid tissue-culture-like areas with extravasation of erythrocytes. Dense, keloid-like collagen (n = 7) and occasional giant cells were seen (n = 6). Mitotic figures (without atypical forms) were readily identifiable (mean 4/10 HPFs). By immunohistochemical staining, the MF were reactive with vimentin, actins, and calponin, while the histiocytes were reactive with CD68. All patients had surgical excision. One patient developed local recurrence (12 months later). All were alive and disease free at last follow-up, with a mean 133 months of follow-up. The principle differential diagnoses include fibrosarcoma, fibromatosis, pleomorphic adenoma, myoepithelioma, neurofibroma, schwannoma, solitary fibrous tumor, leiomyoma, fibrous histiocytoma and myxoma. NF of the parotid gland occurs in middle-aged patients who present with a mass (mean 2.2 cm) in the parotid gland of short duration (1.9 months). FNA misinterpretation frequently leads to excision. Separation from myoepithelial and mesenchymal lesions affecting the parotid gland results in appropriate management.
Sinonasal Tract Neurofibroma: A Clinicopathologic Series of 12 Cases with a Review of the Literature
2015-09-01 03:00:00 AM
Abstract
Neurofibroma (NF), a benign peripheral nerve sheath tumor, is very uncommon in the sinonasal tract, with only a few reported cases in the English literature. Cases within the files of the authors' institutions confined to the sinonasal tract were compared to cases reported in the English literature (Medline 1966–2014). The 12 patients included 6 females and 6 males, aged 26–75 years (mean 46.2 years). The patients usually presented clinically with a mass lesion (n = 11), obstruction (n = 4) or pain (n = 3), with an average symptom duration of 42.9 months. Two patients had neurofibromatosis (NF1). Tumors involved the nasal cavity alone (n = 8), maxillary sinus alone (n = 2), or mixed sites (n = 2), with a range of 0.4–4.1 cm (mean 2.2 cm). The tumors were circumscribed, composed of spindled to wavy cells with curvilinear nuclei set in a background of collagenized stroma and mast cells. Nuclear palisading and perivascular hyalinization were not seen. Mitoses were scant. Pleomorphism, necrosis and increased cellularity were absent. By immunohistochemistry, the lesional cells were S100 protein, SOX10 and NFP positive, while CD34 highlighted the perineurium. INI1 was intact, with strong nuclear expression in all cases. All patients had surgical excision without recurrence (mean follow-up 8.6 years). The principle differential diagnoses include schwannoma, perineurioma, fibromatosis, and solitary fibrous tumor. NF of the sinonasal tract occurs in middle-aged patients without a gender predilection, usually with non-specific symptoms present for a long duration. Tumors are relatively large (mean 2.2), and usually affect one site only. Surgery is curative, with only 16.7 % NF1 associated. S100 protein, SOX10 and NFP highlight the Schwann cells, with CD34 highlighting the perineural fibroblasts.
Ectomesenchymal Chondromyxoid Tumor: A Series of Seven Cases and Review of the Literature
2015-09-01 03:00:00 AM
Abstract
Ectomesenchymal chondromyxoid tumors (ECT) are rare, benign, intraoral mesenchymal soft tissue tumors that can be diagnostically challenging. In this study the demographic, clinical, histopathologic, and immunohistochemical features of seven ECTs are examined. The differential diagnosis of this rare neoplasm is discussed and the literature is reviewed.
Angiosarcoma Developing in a Vagal Schwannoma: A Rare Case Report
2015-09-01 03:00:00 AM
Abstract
Angiosarcoma arising in a schwannoma is extremely rare with only fourteen cases having been reported in the literature to the best of our knowledge. Amongst these fourteen, only five cases developed from vagal schwannoma. We describe a case of epithelioid angiosarcoma arising in a long standing vagal schwannoma in a 41 years male patient. Grossly the tumor was well encapsulated with variegated cut surface. On microscopy the tumor had two distinct components composed of benign schwannoma and malignant angiosarcoma which were further confirmed by immunohistochemistry. On further work up, he was found to have multiple distant metastases. This is the sixth reported case of angiosarcoma arising in a vagal schwannoma. The proposed histogenesis of this rare transformation, its prognostic factors and a review of literature regarding this entity is discussed.
Gene Expression Characterization of HPV Positive Head and Neck Cancer to Predict Response to Chemoradiation
2015-09-01 03:00:00 AM
Abstract
Human papillomavirus (HPV) has been shown to have a causal role in the development of head and neck squamous cell carcinoma. While HPV-positive head and neck cancer is associated with a better response to treatment in the majority of patients, there is a subset who does not respond favorably to current therapy. Identification of these patients could prevent unnecessary morbidity and indicate the need for alternative therapeutic options. Tissue samples were obtained from 19 patients with HPV-positive head and neck squamous carcinoma treated with chemoradiation therapy. HPV status was confirmed by polymerase chain reaction analysis through detection of HPV16 E7 in both DNA and RNA. RNA was isolated from tissue samples and subjected to microarray gene expression analysis. In addition to identification of potential genetic biomarkers (including LCE3D, KRTDAP, HMOX1, KRT19, MDK, TSPAN1), differentially expressed genes associated with genomic stability, cell cycle, and DNA damage were detected between responders and non-responders. These results were further validated with publicly available gene expression studies. This pilot study suggests prospective biomarkers that predict response to therapy. The importance of genes involved with genomic stability is highlighted in both development and progression of head and neck squamous cell carcinoma but also recurrence. Potential development of an assay may prove beneficial to clinicians, assisting them to provide alternative care sooner thus lowering morbidity.
Epidermal Growth Factor Receptor Expression in Spindle Cell Carcinomas of the Head and Neck
2015-09-01 03:00:00 AM
Abstract
Spindle cell carcinoma (SpCC) is an uncommon head and neck squamous cell carcinoma (SCC) variant consisting of spindled and/or pleomorphic cells with epithelial differentiation. Epidermal growth factor receptor (EGFR) is expressed by >90 % of conventional SCC, and high level expression is associated with a poorer prognosis. Anti-EGFR therapies are commonly used to treat head and neck SCC. However, no studies have evaluated EGFR expression in SpCC. Cases of SpCC were retrieved from department files. The diagnosis required either a biphasic lesion with a squamous neoplastic component, or a purely spindle cell or pleomorphic tumor with immunohistochemical positivity for epithelial markers. EGFR immunohistochemistry was performed and was quantified in quartiles. Medical records were reviewed for clinical follow up information. EGFR was expressed in 21/30 (70 %) cases, including in the squamous component in 18/19 (95 %) and the spindle cell component in only 12/30 (40 %). Where the spindle cell component was positive, the intensity and distribution were lower than for the squamous component. Recurrent tumors were predominantly (80–90 %) of the spindle cell component, and had low (or absent) EGFR expression. Kaplan–Meier survival analysis showed no statistically significant differences in overall or disease free survival between the EGFR expressing and non-expressing groups (p = 0.414 and 0.19, respectively). SpCCs of the head and neck have a poor prognosis, and markedly reduced EGFR expression. EGFR-specific therapies may not be ideal for SpCC patients, which may lack EGFR expression, but further studies are needed.
Papillary Cystadenoma of Minor Salivary Glands: Report of 11 Cases and Review of the English Literature
2015-09-01 03:00:00 AM
Abstract
Papillary cystadenoma is a rare, benign salivary gland tumor which is well-circumscribed, containing cystic cavities with intraluminal papillary projections. Only 19 cases arising within minor salivary glands (MnSG) from the oral cavity sites have been reported in the English literature (PubMed 1958–2014). We report 11 new cases of MnSG papillary cystadenomas in conjunction with a review of the literature. Demographic information, clinical and histologic features, treatment and prognosis are compiled and discussed for all 30 cases reported in the English literature.
Juvenile Active Ossifying Fibroma
2015-09-01 03:00:00 AM
Abstract
A case of juvenile active ossifying fibroma affecting a 27 year-old man will be discussed. The characteristic radiologic and histologic features of the entity will be described.
Functional Histology of Salivary Gland Pleomorphic Adenoma: An Appraisal
2015-09-01 03:00:00 AM
Abstract
The complex microstructure of salivary gland pleomorphic adenoma is examined in relation to function. Events related to secretion of macromolecules and absorption, responses to the altered microenvironment and controversies concerning epithelial–mesenchymal transition versus modified myoepithelial differentiation are explored. Their effects on tumor cell phenotypes and arrangements are emphasized. Heterotopic differentiation and attempts at organogenesis are also considered. The approach allows interpreting microstructure independently of histogenetic perceptions, envisaging the tumor cells as a continuum, endorsing luminal structures as the principal components, and defining pleomorphic adenoma as a benign epithelial tumour characterized by variable epithelial–mesenchymal transition, secretion/differentiation and metaplasia.
Undifferentiated Laryngeal Carcinoma with Pagetoid Spread
2015-08-21 03:00:00 AM
Abstract
Pagetoid spread, is used to define intraepithelial spread of cancer cells, when a massive carcinoma is identified beneath the basal membrane. There are only few reports of pagetoid spread at the head and neck region. Herein a 74 year old male patient with bilateral transglottic laryngeal high grade malignant epithelial tumor with pagetoid spread is presented. The tumor was located at the submucosa and there was spread of the CK7 and CK19 positive tumor cells into the non neoplastic mucosa, which was CK5/6 positive, sparing the basement membrane, creating a typical pagetoid pattern. Radiographic and positron emission tomography scan examination of the patient was unremarkable at presentation other than the laryngeal and neck lesions; but extensive systemic metastasis developed at 6 months following operation. To the best of our knowledge no epithelial malignancy with pagetoid spread was described at the larynx. Pagetoid spread may be a hallmark of very aggressive behavior in laryngeal carcinoma.
Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature
2015-08-15 03:00:00 AM
Abstract
Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.
Primary Xanthoma of the Mandible: Report of a Rare Case
2015-08-08 03:00:00 AM
Abstract
Xanthoma is a lesion most commonly seen in soft tissues such as the skin, subcutis, or tendon sheaths. Xanthoma formation is often associated with primary or secondary hyperlipidemia. Primary bone xanthomas are extremely rare benign bone lesions not associated with hyperlipidemia, histopathologically characterized by histiocytes, abundant lipid containing macrophages (foam cells), and multinucleated giant cells. Cholesterol clefts can be found in the medullary bone. Less than ten cases of xanthoma in the mandible have been reported. We present a rare primary intrabony xanthoma in a normolipidemic patient.
Clinicopathologic and Immunophenotypic Characterization of 25 Cases of Acinic Cell Carcinoma with High-Grade Transformation
2015-08-06 03:00:00 AM
Abstract
Acinic cell carcinoma (AiCC) with high-grade transformation is a rare variant of AiCC composed of both a conventional low-grade (LG) AiCC and a separate high-grade (HG) component. We describe here, the clinicopathologic and immunohistochemical features of 25 cases diagnosed between 1990 and 2015. Available tissue was analyzed and compared with a cohort of pure LG AiCC for the morphologic and immunophenotypic profile. Incidence was higher in females (1.8:1) than males with an overall mean age at presentation of 63.2 years. All tumors occurred in the parotid gland including 76 % with facial nerve trunk and branches involvement. Most patients were treated with extensive resection and adjuvant therapy. Local recurrence or distant metastasis occurred in most patients, with 72.7 % dead with disease (mean 2.9 years) and 3 patients alive with disease (mean 2.4 years). The majority of the tumors were composed of a LG microcystic AiCC and a HG component consisting of invasive lobules of undifferentiated cells with predominantly solid, cribriform, and glandular patterns. Acinic differentiation was still present in HG areas but aggressive features such as perineural invasion (76 %), lymphovascular invasion (62 %), positive margins (72 %), high mitotic rate, atypical mitoses and/or comedonecrosis (86 %) were easily identified. Compared to the pure LG AiCC, the cases with HG transformation showed significantly increased expression of cyclin-D1, p53 and Ki-67. Most HG areas of AiCC expressed membranous β-catenin (92 %) and were negative for p63 (three cases were focally positive), S100, SMA, androgen, and estrogen receptors. DOG1 expression was present in all LG AiCC tested with retained expression in 91 % of cases with HG transformation, supporting acinic differentiation in the HG foci. Recognition of AiCC with high-grade transformation is imperative as more aggressive clinical management is warranted.
Κυριακή 23 Αυγούστου 2015
Head and Neck Pathology
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