Eosinophilic angiocentric fibrosis of the nose and sinuses

2014 ◽  
Vol 128 (12) ◽  
pp. 1071-1077 ◽  
Author(s):  
J Rimmer ◽  
P Andrews ◽  
V J Lund
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Case Series ◽  
Rare Condition ◽  
Surgical Excision ◽  
Upper Respiratory Tract ◽  
Immunoglobulin G4 ◽  
Disease Spectrum ◽  
Complete Surgical Excision ◽  
Male Patients ◽  
Eosinophilic Angiocentric Fibrosis

AbstractBackground:Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.Methods:We report the largest single-institution case series of sinonasal eosinophilic angiocentric fibrosis to date, comprising nine patients. The current literature is reviewed, showing emerging evidence that this condition may belong to the immunoglobulin G4-related disease spectrum.Results:The series comprised five female and four male patients, with a mean age at presentation of 53 years. All were treated surgically. Six patients had no signs of recurrent disease after an average of 8.5 years. One patient went on to develop granulomatosis with polyangiitis (Wegener's granulomatosis), which required immunosuppressive therapy.Conclusion:The first-line management of this rare condition is complete surgical excision. Chronic granulomatous conditions, including granulomatosis with polyangiitis, should be excluded before a diagnosis is made, and patients should be carefully followed.

Immunoglobulin G4 related systemic sclerosing disease involving the temporal bone

2010 ◽  
Vol 124 (10) ◽  
pp. 1106-1110 ◽  
Author(s):  
L Masterson ◽  
M Martinez Del Pero ◽  
N Donnelly ◽  
D A Moffat ◽  
E Rytina
Keyword(s):  
Temporal Bone ◽  
Plasma Cells ◽  
Rare Condition ◽  
Immunohistochemical Analysis ◽  
Surgical Excision ◽  
Serum Levels ◽  
Inflammatory Myofibroblastic Tumour ◽  
Inflammatory Pseudotumour ◽  
Immunoglobulin G4 ◽  
Anaplastic Lymphoma

AbstractObjective:To report a rare condition affecting the temporal bone. Immunoglobulin G4 related systemic sclerosing disease is a recently described autoimmune condition with manifestations typically involving the pancreas, biliary system, salivary glands, lungs, kidneys and prostate. Histologically, it is characterised by T-cell infiltration, fibrosis and numerous immunoglobulin G4-positive plasma cells. This condition previously fell under the umbrella diagnosis of inflammatory pseudotumour and inflammatory myofibroblastic tumour.Case report:We present the case of a 58-year-old woman with multiple inflammatory masses involving the pharynx, gall bladder, lungs, pelvis, omentum, eyes and left temporal bone, over a seven-year period. We describe this patient's unusual clinical course and pathological features, which resulted in a change of diagnosis from metastatic inflammatory myofibroblastic tumour to immunoglobulin G4 related systemic sclerosing disease. We also review the literature regarding the management of inflammatory pseudotumours of the temporal bone, and how this differs from the management of immunoglobulin G4 related systemic sclerosing disease.Conclusion:We would recommend a full review of all histological specimens in patients with a diagnosis of temporal bone inflammatory pseudotumour or inflammatory myofibroblastic tumour. Consideration should be given to immunohistochemical analysis for anaplastic lymphoma kinase and immunoglobulin G4, with measurement of serum levels of the latter. Management of the condition is medical, with corticosteroids and immunosuppression, rather than surgical excision.

Keratoacanthoma of the Head and Neck

1980 ◽  
Vol 89 (1) ◽  
pp. 72-74 ◽  
Author(s):  
Richard E. Goodwin ◽  
George H. Fisher
Keyword(s):  
Squamous Cell Carcinoma ◽  
Cell Carcinoma ◽  
Head And Neck ◽  
Squamous Cell ◽  
Surgical Excision ◽  
Clinical Entity ◽  
Diagnosis And Management ◽  
Difficult Diagnosis ◽  
Disease Spectrum ◽  
Complete Surgical Excision

Four representative cases of keratoacanthoma of the head and neck are presented; one of which contained squamous cell carcinoma at its base. Although keratoacanthoma has become recognized as a distinct clinical entity, its similarities to squamous cell carcinoma frequently result in difficult diagnosis. A discussion of the disease spectrum, diagnosis and management is presented. Prompt and complete surgical excision of head and neck keratoacanthoma is recommended.

scholarly journals Cervical root schwannoma: a case series

2016 ◽  
Vol 2 (1) ◽  
pp. 43
Author(s):  
Harshad Nikte ◽  
Nitish Virmani ◽  
Jyoti Dabholkar
Keyword(s):  
Case Series ◽  
Surgical Excision ◽  
Needle Aspiration ◽  
Neck Mass ◽  
Malignant Degeneration ◽  
Spinal Root ◽  
Nerve Tumor ◽  
Complete Surgical Excision ◽  
Operative Findings ◽  
Cervical Root

Cervical root schwannoma is an infrequent benign peripheral nerve tumor though those arising from high cervical spinal root are common amongst the spinal schwannomas. This mass commonly presents as a slow growing, asymptomatic, solitary neck mass with rare potential of   malignant degeneration. Displacement of Internal jugular vein and carotids anteriorly is frequently seen though abutment of esophagus is uncommon. Pre operative diagnosis can be established with imaging and Fine needle aspiration cytology (FNAC). Complete Surgical excision is the treatment of choice. Horner’s syndrome is the most common post operative neurological manifestation. Here we have recorded clinical features intra-operative findings and surgical histopathology. We have described a case of 19 year old male with cellular schwannoma of cervical sympathetic chain with intraspinal extension and arising from C5-C8 level. Another case of cervical root schwannoma is described in a 5 year old girl arising at C4-C5 level. Complete surgical excision was done for both patients with no postoperative neurological affection. The clinico-pathological evaluation and management are described

scholarly journals Upper Cervical Bronchogenic Cyst: A Rare Condition at a Rare Location

2018 ◽  
Vol 09 (01) ◽  
pp. 149-151
Author(s):  
Ranjeet Kumar Jha ◽  
Chandan B. Mohanty ◽  
Chandrashekhar E. Deopujari ◽  
Salman Tehran Shaikh
Keyword(s):  
Bronchogenic Cyst ◽  
Rare Condition ◽  
Surgical Excision ◽  
Cord Compression ◽  
Complete Relief ◽  
Resonance Imaging ◽  
Complete Surgical Excision ◽  
Upper Cervical ◽  
Rare Location ◽  
Intradural Extramedullary

ABSTRACTIntraspinal bronchogenic cyst (SBC) is a rare but important cause of spinal cord compression, commonly seen in the cervicothoracic spine. We report a case of a 43-year-old male, presenting with complaints of neck pain, radiating to right shoulder, with numbness of right hand and fingers. Magnetic resonance imaging of the cervical spine revealed an intradural extramedullary, ventral cystic lesion extending from C2 to C4 vertebral levels. Complete surgical excision was performed, and the patient had a complete relief of symptoms postoperatively. Only 11 cases of SBCs have been reported in literature. We discuss the peculiar location of this lesion, possible embryological reasons and the overall surgical outcome of SBC.

scholarly journals New Treatment Options for Pituitary Granulomatosis With Polyangiitis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A594
Author(s):  
Hessa Boharoon ◽  
Majid AlAmeri ◽  
Abdulla Mohamed Alnuaimi ◽  
Nigel Mendoza ◽  
Stephen McAdoo ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Guidelines ◽  
Dynamic Assessment ◽  
Treatment Options ◽  
Case Series ◽  
Rare Condition ◽  
High Dose ◽  
Pituitary Mass ◽  
Remission Maintenance ◽  
History Of

Abstract Background: Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland and has been reported in 1% of all cases of GPA. Most frequently, it presents with pituitary mass effect and diabetes insipidus (DI). To date, there are no treatment guidelines for this rare condition. Case Presentation: Case 1: A 55 year old female with a history of ANCA-positive pulmonary GPA, previously treated with glucocorticoids and immunosuppressants, presented two years later with cranial DI and bitemporal hemianopia. MRI showed a large sellar mass with suprasellar extension. High dose glucocorticoids resulted in good clinical and radiological response. Further treatment consisted in a combination of Cyclophosphamide and Rituximab (RTX). Further doses of RTX are planned aiming for a period of B-cell depletion. Case 2: A 38 year old female, presented with polyuria, recurrent nosebleeds, headaches and a left visual field defect. Pituitary profile revealed ACTH deficiency and MRI showed a heterogenous cystic lesion with peripheral enhancement and stalk thickening. Steroid replacement led to immediate improvement in her symptoms. Cranial DI was confirmed and raised Proteinase 3 (PR3) antibody suggested GPA. A combination of prednisolone and Methotrexate led to significant improvement of MRI appearances and declining PR3 antibody levels. For remission maintenance, two cycles of RTX were given with further radiological and biochemical improvement, and, following dynamic assessment of her HPA axis, she could be fully weaned off steroids. Case 3 is a 47 year old female with a history of childhood asthma. She was found to have cavitating lung lesions. ANCA positivity confirmed GPA and she was commenced on high-dose steroids. During follow-up, she developed headache, polyuria and polydipsia. MRI pituitary showed a suprasellar lesion and pituitary biopsy revealed inflammatory hypophysitis. Cranial DI was confirmed by water deprivation testing. Previous allergic reactions to both RTX and Ofatumumab precluded anti-CD40 monoclonal antibodies and she was commenced on Azathioprine. A further recurrence of pituitary GPA necessitated escalation of the steroid dose and switch of azathioprine to mycophenolate mofetil. She remains in remission and her steroids reduced to a maintenance dose. Conclusion: GPA pituitary has been observed to occur at variable time after diagnosis often in the absence of any other systemic features. A combination of glucocorticoids and RTX has been approved for severe relapsing pulmonary GPA, however, limited data is available for pituitary GPA. In this case series, the response to high dose steroids and RTX for remission maintenance has been encouraging. Experience with ‘conventional’ immunosuppresants remains limited and therapeutic responses remain variable. Further clinical studies are required to establish effective treatment for pituitary GPA.

Granulomatosis with polyangiitis as a differential diagnosis.

2018 ◽  
Vol 2 (1) ◽  
Author(s):  
Anna Luiza De Araujo Neurauter ◽  
Noemí Andrade Albán
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Acute Otitis Media ◽  
Rare Condition ◽  
Pulse Therapy ◽  
Upper Respiratory Tract ◽  
Conjunctival Hyperemia ◽  
Small Vessels ◽  
Recurrent Acute Otitis Media ◽  
Clinical Stability

Granulomatosis with polyangiitis, a small vessels vasculitis, involves mostly upper respiratory tract, lungs and kidneys. It’s a multisystemic pathology, with no sex predominance, affecting adults over 40 years old. Case report: K.S.D, 64 years, female, came to emergency room with dyspnea, cough and coryza, 15 days after a recurrent acute otitis media surgical treatment. A CT showed bilateral pleural effusion, consolidation and a lung mass, being admitted in Intensive Care Unit. Subsequently, developed mastoiditis and conjunctival hyperemia and used several antibiotic therapys. Patient was discharged 40 days later, with clinical improvement. One month later, was admitted again with pulmonary septic shock, in addition to sinusitis, polyarthralgia, purpura and acute renal injury, requiring hemodyalisis. Biomarkers were requested, such as c-ANCA and rheumatoid factor, with positive result and antinuclear factor, with negative result. A renal biopsy also has been solicitated, showing glomerulonephritis. The diagnosis was granulomatosis with polyangiitis, treated with methylprednisone pulse therapy and cyclophosphamide, progressing to clinical stability and receiving discharge with outpatient follow-up. Conclusion: Granulomatosis with polyangiitis is a rare condition, with that should be considered when patient presents the triad: upper and lower airways and glomerulonephritis. Early diagnosis and immunosuppressive therapy are essential to reduce its morbimortality. Recibido: 18-11-2017 / Aceptado: 25-01-2018

scholarly journals Unusual Presentation of a Rare Tumor of the Dorsal Surface of the Foot

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
O. Hocar ◽  
H. Yacoubi ◽  
N. Akhdari ◽  
S. Amal ◽  
F. Ait Essi ◽  
...  
Keyword(s):  
Dorsal Surface ◽  
Rare Condition ◽  
Surgical Excision ◽  
Good Prognosis ◽  
Accurate Diagnosis ◽  
Unusual Presentation ◽  
Benign Condition ◽  
Complete Surgical Excision ◽  
Soles Of The Feet ◽  
Painless Mass

Calcifying aponeurotic fibroma (CAF) was originally described by Keasbey in 1953 as juvenile aponeurotic fibroma, most commonly occurring in the palms of the hands and soles of the feet in children and adolescents. It usually presents as a firm, painless mass without preceding trauma. We report a case of this rare condition with an unusual presentation in a 60-year-old woman affecting the dorsal surface of the foot. It is a relatively benign condition with a good prognosis following complete surgical excision. It may have a slightly increased incidence in males. The accurate diagnosis is based only on histology but it is essential to differentiate it from other sinister lesions such as fibrosarcoma that may lead to amputation.

Primary extramedullary plasmacytoma of the parotid gland: a case report and review of the literature

1998 ◽  
Vol 112 (2) ◽  
pp. 179-181 ◽  
Author(s):  
J. Gonzalez-Garcia ◽  
K. Ghufoor ◽  
G. Sandhu ◽  
P. A. Thorpe ◽  
J. Hadley
Keyword(s):  
Parotid Gland ◽  
Rare Condition ◽  
Surgical Excision ◽  
Good Prognosis ◽  
Extramedullary Plasmacytoma ◽  
Treatment Modalities ◽  
Malignant Neoplasms ◽  
Upper Respiratory Tract ◽  
Review Of The Literature ◽  
The Right

AbstractSolitary extramedullary plasmacytomas are uncommon malignant neoplasms with a great predilection for the upper respiratory tract. Extramedullary plasmacytoma involving the parotid gland is an extremely rare condition. This report describes the case of a primary extramedullary plasmacytoma arising in the right parotid gland of a 63-year-old man. A review of the literature reveals that this disease has generally a good prognosis, provided that multiple myeloma has been excluded. The treatment of choice should be surgical excision followed by radiotherapy, although from this study there is insufficient evidence to suggest that this is more effective than surgery alone. All patients must have regular long-term follow-up to monitor for recurrence or dissemination, in which event chemotherapy may be considered in addition to the other treatment modalities.

Loss of Reflex Tearing: An Expected Consequence of Juvenile Nasopharyngeal Angiofibroma

2005 ◽  
Vol 133 (4) ◽  
pp. 605-610 ◽  
Author(s):  
Alexander T. Hillel ◽  
Rebecca C. Metzinger ◽  
Andrew J. Nemechek ◽  
Daniel W. Nuss
Keyword(s):  
Surgical Treatment ◽  
Hospital Setting ◽  
Case Series ◽  
Surgical Excision ◽  
Juvenile Nasopharyngeal Angiofibroma ◽  
Retrospective Case ◽  
Nasopharyngeal Angiofibroma ◽  
Complete Surgical Excision ◽  
Major Teaching Hospital ◽  
Pterygopalatine Ganglion

OBJECTIVE: To report the loss of reflex tearing after surgical treatment of juvenile nasopharyngeal angiofibroma (JNA). STUDY DESIGN AND SETTING: A retrospective case series of 8 patients with surgical extirpation of JNA from 1995 to 2003 in a major teaching hospital setting was studied for symptomatic xerophthalmia. RESULTS: One patient was lost to follow-up. Four of the remaining 7 patients reported a dry ipsilateral eye after surgical treatment of JNA. CONCLUSION: The location of the pterygopalatine ganglion and its associated fibers in the pterygopalatine fossa is directly adjacent to the location of origin of JNA. Lacrimal innervation passes through the pterygopalatine ganglion. Given the extensive nature of advanced JNA and mandate for complete surgical excision, lacrimal dysfunction should be considered an expected consequence of surgery. SIGNIFICANCE: The loss of reflex tearing has not been reported as a consequence of JNA or its surgical treatment. EBM Rating: C.

scholarly journals Benign Multicystic Peritoneal Mesothelioma: A Rare Condition in an Uncommon Gender

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Muhammad S. Khurram ◽  
Hamadullah Shaikh ◽  
Uqba Khan ◽  
Jacob Edens ◽  
Warda Ibrar ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Intraperitoneal Chemotherapy ◽  
Hyperthermic Intraperitoneal Chemotherapy ◽  
Case Series ◽  
Premenopausal Women ◽  
Rare Condition ◽  
Peritoneal Mesothelioma ◽  
Previous Surgery ◽  
Mediterranean Fever ◽  
Male Patients

Benign Multicystic Peritoneal Mesothelioma (BMPM) is a rare condition that arises from the abdominal peritoneum. Fewer than 200 cases have been reported worldwide. BMPM usually affects premenopausal women and is extremely rare in men. Many factors are suspected to contribute to its development, such as previous surgery, endometriosis, and familial Mediterranean fever. The main management is surgical resection; however, it is estimated that the recurrence rate is up to 50%. Malignant transformation is rare. We report a case series of three male patients who were diagnosed with BMPM and were treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC).

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