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Orbital neoplasms

Sayaka Yuzawa, Satoshi Tanikawa, Isamu Kunibe, Hiroshi Nishihara, Kazuo Nagashima, Shinya Tanaka
We present a rare case of giant cell-rich solitary fibrous tumor (SFT) arising at the left external auditory canal in a 31-year-old woman. The tumor was well-circumscribed and composed of spindle-shaped cells with abundant collagenous bands. Scattered multinucleate giant cells were observed, some of which lined pseudovascular spaces. Although a focal mild-hypercellular area was observed, mitoses were rare and necrosis was absent. Interstitial mast cells were scattered, especially in the hypercellular area. Immunohistochemically, CD34, vimentin, and Bcl-2 presented diffuse positivity...
October 6, 2016: Pathology International
Erika F Rodriguez, Jaishri Blakeley, Shannon Langmead, Alessandro Olivi, Anthony Tufaro, Abeer Tabbarah, Gail Berkenblit, Justin M Sacks, Scott D Newsome, Elizabeth Montgomery, Fausto J Rodriguez
Leptomeningeal dissemination of low grade Schwann cell neoplasms is an exceptionally rare occurrence, and has not been well documented in the literature. We encountered two cases of leptomeningeal dissemination of low grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with NF1 and a progressive low grade MPNST developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient's death...
September 22, 2016: Human Pathology
Q Zhong, Z G Huang, J G Fang, X J Chen, X H Chen, L Z Hou, P D Li, H Z Ma, S Z He
Objective: To evaluate the outcome of one-stage reconstruction of maxillary and orbital defects with modified temporalis muscle flap (TMF) following the removal of malignant neoplasms. Methods: In this retrospective study, 15 patients underwent the reconstruction of defects of orbital floor and palate after maxillectomy for malignant tumor were included from June 2008 to June 2014. The modified temporalis muscle flap was used to repair the defects after surgery, and functional outcomes were analyzed. Results: All the patients were followed up for 12-81 months...
September 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
Ritesh R Kalaskar, Ashita R Kalaskar
Neuroblastoma is an extremely rare pediatric neoplasm whose prognosis becomes poor and poor as the age advances. It can be sporadic or nonfamilial in origin. It is primarily a tumor of abdominal origin from where it metastasis to lymph nodes, liver, intracranial and orbital sites, and central nervous system. There is no standard dental treatment protocol for the management of neuroblastoma due to its poor survival rate and rarity. However, dental treatment may follow the protocol of preventive and restorative...
July 2016: Contemporary Clinical Dentistry
Aashrai Gudlavalleti, Ryan Dean, Yuxin Liu, Amit S Dhamoon
Sinonasal neuroendocrine tumours (NETs) are rare, aggressive neoplasms with a high recurrence potential. There are no robust protocols for the management of these tumours. An 81-year-old man presented with an incidental sinonasal mass visualised on CT scan of head. Over the next few weeks he developed new onset, progressively worsening headache, right eye ptosis and restricted extraocular movements. Imaging confirmed a rapidly enlarging tumour involving the right nasal cavity and the orbit. Biopsy showed a large cell neuroendocrine carcinoma of the right ethmoidal sinus...
2016: BMJ Case Reports
Jianqin Shen, Hongguang Cui, Shi Feng
PURPOSE: Olfactory neuroblastoma (ONB) is a rare neuroectodermal malignant neoplasm that originates from the olfactory sensory epithelium. In reported cases involving the orbit, the primary neoplasms could invade the orbits through bone destruction or cranio-orbital communication anatomical structure. The present study reports a rare case of orbital metastatic tumor arising from an intracranial ONB in a Chinese patient, which is the first case demonstrating an uncommon route of ONB involving the orbit...
2016: OncoTargets and Therapy
Alex Muturi, Vihar Kotecha, Cynthia Ojee, Desmond Mang'oka, John Muthuri
BACKGROUND: Inflammatory pseudotumour refers to a non-malignant tumour-like mass resulting from an inflammatory reaction that is composed of granulation tissue with leukocyte infiltration that commonly occurs in the paediatric or young adult population. These tumours occur more commonly in the lungs and the orbit but rarely does it affect the gastrointestinal tract. It poses a clinical diagnostic challenge since it is a benign condition than can mimic the malignant counterpart. Our case is a rare presentation of the caecal pseudotumour in the presence of a right undescended abdominal testis evaluated as a caecal tumour with a differential diagnosis of a testicular malignancy...
2016: World Journal of Surgical Oncology
Adam R Sweeney, Divakar Gupta, C Dirk Keene, Patrick J Cimino, Christopher B Chambers, Shu-Hong Chang, Eissa Hanna
Peripheral nerve sheath tumors of the orbit and ocular adnexa are a rare group of neoplasms hallmarked by nonspecific clinical presentations, variable tumor locations, challenging therapeutic efforts, and occasional diagnostic dilemmas. We review these tumor types and provide an updated summary on their clinical, histopathologic, radiological, and emerging molecular features.
August 25, 2016: Survey of Ophthalmology
N Sood, N Sehrawat
Rhabdomyosarcoma (RMS) is a fast growing, malignant tumour arising from immature mesenchymal cells, committed to skeletal muscle differentiation. It is more often seen in the paediatric population and constitutes less than 1% of all malignancies and less than 3% of all soft tissue tumours. RMS of the paranasal sinuses constitutes 10-15% of adult head and neck RMS, ethmoidal and maxillary sinuses being the most common. We report a 56-year-oldman presenting with left nasal obstruction, epistaxis on and off and left cheek swelling...
August 2016: Malaysian Journal of Pathology
Aaron D Skolnik, Laurie A Loevner, Deepak M Sampathu, Jason G Newman, John Y Lee, Linda J Bagley, Kim O Learned
Schwannomas are benign nerve sheath tumors that may arise along the complex course of the cranial nerves (CNs), anywhere in the head and neck. Sound knowledge of the CN anatomy and imaging features of schwannomas is paramount for making the correct diagnosis. In this article, we review approaches to diagnosing CN schwannomas by describing their imaging characteristics and the associated clinical presentations. Relevant anatomic considerations are highlighted by using illustrative examples and key differential diagnoses categorized according to regions, which include the anterior skull base, orbit, cavernous sinus, basal cisterns, and neck...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Yu Yu Thar, Poras Patel, Tiangui Huang, Elizabeth Guevara
Small cell neuroendocrine carcinoma (SNEC) is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach...
2016: Case Reports in Oncological Medicine
Munehito Moriyama, Satoru Kodama, Takashi Hirano, Masashi Suzuki
Solitary fibrous tumor (SFT) is an uncommon neoplasm that usually arises from the pleura. Recently, SFTs have been reported in the head and neck region located in subsites such as the orbit. SFTs of the lacrimal sac are extremely rare, with only six cases reported in the English literature. We describe a SFT arising from the right lacrimal sac and extending along the nasolacrimal duct into the nasal cavity. Although, the tumor could not be removed by endoscopic-modified medial maxillectomy (EMMM) alone, combined approach with EMMM and a small external incision achieved the complete removal of the tumor...
August 2, 2016: Auris, Nasus, Larynx
Ying Zhu, Miao Fan, Sagar Pandey, Wei Liang, Dandan Chang
INTRODUCTION: Epithelioid hemangioedothelioma (EHE) is a rare vascular tumor characterized by neoplastic proliferation of epithelioid or histiocytoid epithelial cells. EHE of bone constitutes <1% of primary malignant bone tumor. EHE in the skull is an extremely rare case. Here, we report a case of multiple neoplasm of the skull in a 15-month-old boy who presented with gradual facial swelling for 2 months. On computed tomography (CT) scan, multiple irregular osteolytic lesions were seen on the right maxillary, sphenoid, left zygoma, and roof of the left orbit...
July 2016: Medicine (Baltimore)
I Schmack, H E Grossniklaus, S Hartmann
Lymphomas of the ocular adnexa and intraocular tissue include a wide range of lymphoproliferative neoplastic disorders. They are predominantly extranodal non-Hodgkin lymphomas (NHL). The World Health Organization (WHO) classification of lymphoid neoplasm and individual morphological, immunophenotypical, and molecular genetic features, indicate that they may be divided into B-cell (approximately 80 % of all NHL) and T-cell lymphomas (approximately 10-20 % of all NHL). The most common forms of ocular NHL are extranodal marginal zone lymphoma (EMZL) of the mucosa-associated lymphoid tissue (MALT-type), follicular lymphoma (FL), diffuse large B-cell lymphoma, and mantel cell lymphoma...
July 2016: Klinische Monatsblätter Für Augenheilkunde
Mary Beth Cunnane, Hugh David Curtin
Diseases of the orbit can be categorized in many ways, but in this chapter we shall group them according to etiology. Inflammatory diseases of the orbits may be infectious or noninfectious. Of the infections, orbital cellulitis is the most common and typically arises as a complication of acute sinusitis. Of the noninfectious, inflammatory conditions, thyroid orbitopathy is the most common and results in enlargement of the extraocular muscles and proliferation of the orbital fat. Idiopathic orbital inflammatory syndrome is another cause of inflammation in the orbit, which may mimic thyroid orbitopathy or even neoplasm, but typically presents with pain...
2016: Handbook of Clinical Neurology
D R Shatzkes, L E Ginsberg, M Wong, A H Aiken, B F Branstetter, M A Michel, N Aygun
SMARCB1 (INI1)-deficient sinonasal carcinomas were first described in 2014, and this series of 17 cases represents the first imaging description. This tumor is part of a larger group of SMARCB1-deficient neoplasms, characterized by aggressive behavior and a rhabdoid cytopathologic appearance, that affect multiple anatomic sites. Clinical and imaging features overlap considerably with other aggressive sinonasal malignancies such as sinonasal undifferentiated carcinoma, which represents a common initial pathologic diagnosis in this entity...
July 7, 2016: AJNR. American Journal of Neuroradiology
Teng Yu, Genbo Xu, Xiaohua Xu, Jing Yang, Luyin Ding
Myeloid sarcoma is a type of malignant neoplasm composed of myeloblasts that locates extramedullary. The present study reports the case of a 31-year-old female who presented with upper abdominal pain, melena, vomiting and jaundice. The abdominal computed tomography revealed a mass in gastric antrum area and possible infiltration of the duodenum, gallbladder and head of the pancreas, with possible retroperitoneal lymph node metastasis. The tumor grew quickly and led to serious obstructive jaundice. New masses developed in the bilateral orbits and left breast within 2 months of admission...
June 2016: Oncology Letters
Hayyam Kiratli, Salih Uzun, Ali Varan, Canan Akyüz, Diclehan Orhan
Inflammatory myofibroblastic tumor (IMT) is a distinct mesenchymal neoplasm of myofibroblastic spindle cells associated with an inflammatory infiltrate formed by lymphocytes, eosinophils, and plasma cells in a myxoid or collagenous stroma. This tumor has a predilection for children and young adults and most commonly occurs in the lungs, retroperitoneum, abdomen, and pelvis. Ocular and orbital involvement is exceedingly rare. We describe a case of IMT in a 7-year-old girl involving the cornea, conjunctiva, and the anterior orbit treated with crizotinib, resulting in complete tumor remission...
June 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
V M Matějka, P Mukenšnabl, R Tupý, O Fiala, J Fínek
Goblet cell carcinoid (GCC) of the appendix is extremely rare, representing approximately 5% of all primary appendiceal neoplasms. Histologically there are three groups of GCC: group A (typical GCC), adenocarcinoma ex GCC signet ring cell type (group B), and adenocarcinoma ex GCC poorly differentiated carcinoma type (group C), which is the most aggressive. GCC metastasizes in 15-60% of cases, mainly to the ovaries, pelvis, abdominal cavity, ribs, vertebrae, and lymph nodes. Hematogenous metastasis to the liver or other parenchymal organs can occur, but this is very rare...
2016: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
Dejan M Rašić, Miroslav Knežević, Tatjana Terzić, Gordana Vlajković
A 66-year-old man developed a slowly enlarging, bilateral, painless, periorbital and orbital swelling with ptosis, non-axial proptosis, chemosis, exposure keratopathy and decreased vision in both eyes. He had fever, night sweats, and weight loss (B-symptoms), along with lymphadenopathy and elevated serum lactate dehydrogenase, with no prior history of lymphoma. A transpalpebral incisional biopsy revealed a rare case of mantle cell lymphoma of blastoid variant, stage IVB. The main immunophenotype characteristics were cyclin D1 +, CD5 +, CD10 -, CD23 -, Bcl-6 -/+, and a high (up to 80%) Ki-67 proliferation index...
May 30, 2016: Survey of Ophthalmology
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