Fibromatosis: a potential sequela of neuromuscular choristoma

2012 ◽  
Vol 116 (2) ◽  
pp. 399-408 ◽  
Author(s):  
Marie-Noëlle Hébert-Blouin ◽  
Bernd W. Scheithauer ◽  
Kimberly K. Amrami ◽  
Susan R. Durham ◽  
Robert J. Spinner
Keyword(s):  
Mayo Clinic ◽  
Aggressive Fibromatosis ◽  
Extensive Literature ◽  
Nerve Lesion ◽  
Imaging Studies ◽  
Lower Intensity ◽  
Imaging Characteristics ◽  
Collagenous Tissue ◽  
Muscle Nerve

Object Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion in which mature skeletal muscle fibers lie within the nerve and its fascicles. Given limited follow-up, its natural history is poorly understood. The occurrence of aggressive fibromatosis in one of the authors' patients and its occurrence in reported cases suggests an etiological relationship between the 2 lesions. This study attempts to explain the association and its frequency. Methods All cases of NMCs seen in consultation or treated at the Mayo Clinic were identified. Demographic and clinical data were reviewed in cases with coexistent aggressive fibromatosis. Pathology and neuroimaging studies were reexamined. In addition, an extensive literature review was performed to explore the association of NMC with aggressive fibromatosis, with special attention given to pathological and imaging characteristics and the development of aggressive fibromatosis. Results The authors identified 10 patients with a diagnosis of NMC who were treated at the Mayo Clinic between 1992 and 2010. Four of 5 with adequate follow-up had developed a definite or suspected aggressive fibromatosis. A review of the initial pathological specimens in these cases revealed no evidence of fibromatosis, but all of the lesions exhibited accompanying hypocellular collagenous tissue. On MR images, all cases showed areas of low signal intensity, which significantly differed from muscle, nerve, and NMC components. On available serial MR imaging studies, aggressive fibromatosis seemed to originate in such lower-intensity regions. In the 18 previously reported cases of NMC, 5 patients developed recurrent masses diagnosed as either definite (2 cases) or possible (3 cases) fibromatosis. Review of the published imaging studies in these cases suggests the presence of lower intensity areas similar to those observed in the 10 patients treated at the Mayo Clinic. Conclusions This study confirms that the development of aggressive fibromatosis in patients with NMC has been underreported. A direct relationship between the NMC and the development of aggressive fibromatosis is suggested by pathological and neuroimaging evidence.

A Tour of the Thymus: A Review of Thymic Lesions with Radiologic and Pathologic Correlation

2015 ◽  
Vol 66 (1) ◽  
pp. 5-15 ◽  
Author(s):  
Alan J. Goldstein ◽  
Isabel Oliva ◽  
Hedieh Honarpisheh ◽  
Ami Rubinowitz
Keyword(s):  
Imaging Studies ◽  
Dynamic Changes ◽  
Cross Sectional ◽  
Radiographic Diagnosis ◽  
Imaging Characteristics ◽  
Pathologic Features ◽  
Cross Sectional Imaging ◽  
Pathologic Lesions ◽  
Periods Of Stress

The thymus is routinely encountered on cross-sectional imaging studies of the chest. It has a variable appearance, undergoes dynamic changes during periods of stress, and demonstrates numerous different pathologic lesions. Understanding the imaging characteristics of these different lesions facilitates accurate radiographic diagnosis and can prevent unnecessary follow-up imaging and intervention. This article will review normal thymic anatomy and development, thymic hyperplasia and associated medical conditions, and the imaging and pathologic features of various benign and malignant thymic lesions.

Intracranial parenchymal schwannoma

1993 ◽  
Vol 79 (2) ◽  
pp. 217-222 ◽  
Author(s):  
Gian Piero Casadei ◽  
Takashi Komori ◽  
Bernd W. Scheithauer ◽  
Gary M. Miller ◽  
Joseph E. Parisi ◽  
...  
Keyword(s):  
Parietal Lobe ◽  
Clinical Course ◽  
Cystic Degeneration ◽  
Resonance Imaging ◽  
Imaging Studies ◽  
Content Type ◽  
Imaging Characteristics ◽  
Benign Nature ◽  
Peripheral Enhancement

✓ The clinical, radiological, and pathological features of nine cases of intracranial parenchymal schwannoma are described. The clinical course in four patients 23 years of age or younger mirrored the indolent nature of this neoplasm. Imaging studies included computerized tomography in eight patients and magnetic resonance imaging in three. The lesions were well demarcated with only mild surrounding edema. Five tumors were deep within the temporoparieto-occipital region, three were in the cerebellum, and one lay peripherally in the parietal lobe. Over two-thirds of the nine tumors were either cystic (five) or contained areas of cystic degeneration (two). One lesion was frankly hemorrhagic. A variety of imaging characteristics and contrast enhancement patterns were observed, including those of a cyst with a mural nodule and peripheral enhancement. Of the four solid neoplasms, two enhanced homogeneously while the other two demonstrated heterogeneous enhancement. Six tumors were resected totally. The follow-up period ranging from 2 months to 2 years has shown no recurrences. Microscopically, immunohistochemically, and ultrastructurally, the tumors were indistinguishable from peripheral schwannomas. A possible mechanism underlying the histogenesis of these rare lesions is discussed. The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors such as pilocytic astrocytoma, and favorable response to resection.

Adrenal incidentaloma: evaluation and management

2008 ◽  
Vol 61 (11) ◽  
pp. 1168-1173 ◽  
Author(s):  
P K Singh ◽  
H N Buch
Keyword(s):  
Surgical Intervention ◽  
Adrenal Incidentaloma ◽  
Adrenal Incidentalomas ◽  
Imaging Studies ◽  
Imaging Characteristics ◽  
Risk Of Malignancy ◽  
Life Threatening ◽  
History Of ◽  
Adrenal Masses

Adrenal incidentalomas are adrenal masses discovered incidental to imaging studies performed for reasons unrelated to adrenal pathology. Although most adrenal incidentalomas are non-functioning benign adenomas, their increasing prevalence presents diagnostic and therapeutic challenges. The assessment of adrenal incidentalomas is aimed at deciding whether or not the tumour should be surgically removed. Adrenalectomy is indicated for phaeochromocytoma, other symptomatic hormone-secreting tumours and those with a high risk of malignancy. Biochemical screening for tumour hypersecretion is mandatory in all adrenal incidentalomas, since hormone secreting tumours may be clinically silent. The diagnosis of phaeochromocytoma is of paramount importance because of its life-threatening complications. Non-functioning adrenal incidentalomas need assessment for risk of malignancy, and this is based on the size of the tumour and its imaging characteristics. An observational policy with periodic radiological and biochemical reassessment is pursued in patients with non-functioning incidentalomas with low malignancy risk. The duration and frequency of reassessment remains unclear, as the natural history of adrenal incidentalomas has yet to be clearly defined, and there is a lack of controlled studies comparing surgical intervention with observation. However, the possibility of acquiring autonomous hypersecretion or conversion to malignancy in an incidentaloma diagnosed to be a benign non-functioning lesion is very low, and most patients may be safely discharged after an initial follow-up period of 2 years.

Principles of pediatric lupus nephritis in a prospective contemporary multi-center cohort

Lupus ◽  
2021 ◽  
pp. 096120332110286
Author(s):  
Kathleen M Vazzana ◽  
Ankana Daga ◽  
Beatrice Goilav ◽  
Ekemini A Ogbu ◽  
Daryl M Okamura ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Kidney Function ◽  
Lupus Erythematosus ◽  
Extensive Literature ◽  
Short Term ◽  
Renal Outcomes ◽  
Childhood Arthritis ◽  
Black Patients ◽  
End Stage

Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort. After an extensive literature review, six principles of LN in cSLE were identified. The CARRA registry was queried to evaluate these principles in determining the rate of LN in cSLE, median time from cSLE diagnosis to LN, short-term renal outcomes, and frequency of rituximab as an induction therapy. Of the 677 cSLE patients in the CARRA registry, 32% had documented LN. Decline in kidney function was more common in Black cSLE patients than non-Black patients ( p = 0.04). Black race was associated with worse short-term renal outcomes. In short-term follow up, most children with LN had unchanged or improved kidney function, and end stage kidney disease (ESKD) was rare. Ongoing follow-up of cSLE patients in the CARRA registry will be necessary to evaluate long-term outcomes to inform risk, management, and prognosis of LN in cSLE.

Monoclonal Gammopathy of Undetermined Significance: Indications for Prediagnostic Testing, Subsequent Diagnoses, and Follow-up Practice at Mayo Clinic

2020 ◽  
Vol 95 (5) ◽  
pp. 944-954
Author(s):  
Aishwarya Ravindran ◽  
Kandace A. Lackore ◽  
Amy E. Glasgow ◽  
Matthew T. Drake ◽  
Miriam A. Hobbs ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Monoclonal Gammopathy ◽  
Undetermined Significance

scholarly journals Isolated Tuberculous Tenosynovitis of the Anterior Tibial and Extensor Digitorum Longus Tendons

2013 ◽  
Vol 3 ◽  
pp. 37 ◽  
Author(s):  
Berhan Genç ◽  
Aynur Solak ◽  
Aslan Mayda ◽  
Nazime Şen
Keyword(s):  
Clinical Symptoms ◽  
Granulomatous Inflammation ◽  
Tendon Sheath ◽  
Imaging Studies ◽  
Rare Form ◽  
Common Diagnosis ◽  
Anterior Tibial ◽  
Histopathological Studies ◽  
Tuberculous Tenosynovitis

Musculoskeletal system is involved in 1-5% of extrapulmonary cases of tuberculosis. Tuberculous tenosynovitis is a rare form of musculoskeletal tuberculosis. Tuberculosis of the tendon sheath in the hand has been seen in a few cases. Involvement of the tendons of the leg is less common. Diagnosis is not easy as there are no specific clinical symptoms or signs. A 33-year-old male presented with painful swelling in the distal right lower limb that caused restriction of movement. Imaging studies showed inflammation and infection of the extensor digitorium longus and tibialis anterior tendons. Histopathological studies showed a necrotizing granulomatous inflammation in the synovial tissue. A diagnosis of tuberculosis was made and medical treatment was initiated that proved successful. Patient remained infection-free at 26-month follow-up examination.

scholarly journals Solitary Skeletal Muscle Metastasis as First Site of Recurrence of Cervical Cancer: A Case Report

2017 ◽  
Vol 10 (2) ◽  
pp. 694-698 ◽  
Author(s):  
Indumathy Varadarajan ◽  
Aparna Basu ◽  
Sherri Besmer ◽  
Jaganmohan Poli ◽  
Scott Richard ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Cervical Cancer ◽  
Case Report ◽  
African American Woman ◽  
American Woman ◽  
Extensive Literature ◽  
Upper Arm ◽  
Skeletal Muscle Metastasis ◽  
Muscle Metastasis

Cervical cancer is the fourth most common cancer in women worldwide, with a large majority of prevalence (85%) in developing countries. As of 2012, it accounts for 7.5% of all female cancer deaths. Despite its high prevalence, skeletal muscle metastasis from cervical cancer is extremely uncommon. In our extensive literature search, we were able to find only 8 cases where skeletal muscle metastasis was the only site of recurrence. We report a case of a 52-year-old African-American woman with a past medical history of cervical cancer (stage IIIB) who presented with pain and swelling in her left upper arm over the preceding 2 months. MRI of the left upper arm showed a solid well-circumscribed mass measuring 7.0 × 2.8 × 2.5 cm, deep to the biceps. Biopsy of the mass revealed a metastatic squamous cell carcinoma that was p16-positive. PET scan showed that the lesion was the sole site of metastasis. She received local radiation with concurrent chemotherapy. Follow-up MRI 6 months after the completion of therapy showed resolution of the mass. She has remained disease-free for the last 24 months as evidenced by a PET/CT scan in May 2016. In this case report, we discuss the role of imaging and pathology in the diagnosis of a solitary metastatic lesion. This case also emphasizes the importance of a close follow-up which aids in early intervention, increasing overall survival.

A case of nodular episcleritis mimicking a solitary giant episcleral mass

2021 ◽  
pp. 112067212110528
Author(s):  
Lan Zhou ◽  
Juanjuan Wang ◽  
Guihua Xu ◽  
Dingding Wang ◽  
Xiaoyi Wang ◽  
...  
Keyword(s):  
Topical Steroid ◽  
Inflammatory Cells ◽  
Systemic Hypertension ◽  
Eye Drops ◽  
Definite Diagnosis ◽  
Imaging Studies ◽  
Immunohistochemical Examination ◽  
History Of ◽  
Inflammatory Drugs

Purpose To describe an atypical nodular episcleritis mimicking a solitary giant episcleral mass, which is not attributed to any systemic diseases and identified only after immunohistochemical examination. Case report A sixty-year-old Chinese woman with systemic hypertension presented with 6-month history of giant, solitary and redness epibulbar mass arising from the superior aspect of her left eye. The lesion gradually enlarged, even with 6-month history of irregular topical steroid eye drops treatment. Imaging studies and laboratory test revealed a 10 mm × 8 mm episcleral mass absence of any infection indicator and autoimmune antibody changes. The mass was completely removed before its extension through the deep scleral, histopathologic examination revealed a nodular episcleritis composed of various chronic inflammatory cells infiltration. Topical steroid eye drops treatment combined with oral steroidal anti-inflammatory drugs was then administrated regularly for 1 month, and no recurrence occurred after 1-year follow-up. Conclusion Nodular anterior episcleritis is characterized by underlying chronic inflammation of the anterior episclera and can be presented as asymptomatic episcleral mass. Besides a thorough investigation systemically, tissue biopsy is required for definite diagnosis.

Role of multiparametric MRI in long-term surveillance following focal laser ablation of prostate cancer

2021 ◽  
pp. 20210414
Author(s):  
Mark Paxton ◽  
Eitan Barbalat ◽  
Nathan Perlis ◽  
Ravi J Menezes ◽  
Mark Gertner ◽  
...  
Keyword(s):  
Laser Ablation ◽  
Recurrent Disease ◽  
Small Sample Size ◽  
Focal Therapy ◽  
Outcome Data ◽  
Small Sample ◽  
Restricted Diffusion ◽  
Treatment Site ◽  
Imaging Characteristics

Objective: Determine the multiparametric magnetic resonance imaging (mpMRI) appearance of the prostate following focal laser ablation (FLA) for PCa and to identify imaging characteristics associated with recurrent disease. Methods: Retrospective analysis of patients who underwent FLA for low-intermediate risk PCa between 2010 and 2014 was performed. Early (median 4 months) and late mpMRI (median 49 months) follow-up were qualitatively assessed for T2-weighted, dynamic contrast enhanced (DCE) and diffusion weighted imaging (DWI) appearances and also compared to corresponding PSA values and biopsy results. Results: 55 cancers were treated in 54 men (mean age 61.0 years). Early mpMRI was performed in 30 (54.5%) patients while late follow-up mpMRI in 42 (84%). Ill-defined scarring with and without atrophy at the treatment site were the most common appearances. In patients with paired MRI and biopsy, one of four patients with clinically significant PCa on biopsy (≥GG2 or≥6 mm GG1) showed hyperenhancement or restricted diffusion at early follow-up. At late follow-up, positive biopsies were seen in 5/8 (63%) cases with hyperenhancement and 5/6 (83%) cases with restricted diffusion at the treatment site. PSA change was not associated with biopsy results at either time point. Conclusion: mpMRI is able to document the morphological and temporal changes following focal therapy. It has limited ability to detect recurrent disease in early months following treatment. Late-term mpMRI is sensitive at identifying patients with recurrent disease. Small sample size is, however, a limitation of the study. Advances in knowledge: Implementing MRI in follow-up after FT may be useful in predicting residual or recurrent PCa and therefore provide reliable outcome data.

scholarly journals Radiation therapy for pediatric craniopharyngioma

2010 ◽  
Vol 28 (4) ◽  
pp. E10 ◽  
Author(s):  
Erin N. Kiehna ◽  
Thomas E. Merchant
Keyword(s):  
Radiation Therapy ◽  
Disease Control ◽  
Conservative Surgery ◽  
Tumor Control ◽  
Extensive Literature ◽  
Long Term Effects ◽  
Secondary Tumors ◽  
Limited Surgery

Object The treatment of craniopharyngioma is highly controversial. Continued advances in limited surgery and radiation therapy have maintained excellent local disease control while minimizing treatment-related sequelae. Further analyses of outcomes are necessary to characterize the long-term effects of radiation therapy. Methods An extensive literature review was performed for all studies including radiation therapy, with or without surgery, for pediatric craniopharyngioma. Results The authors identified 32 papers describing radiation therapy for treatment of pediatric craniopharyngioma, with disease control ranging from 44 to 100%. Modern studies report at least 90% disease control with 5-year follow-up. Fifteen studies reported outcomes, demonstrating that more than two-thirds of patients treated with surgery and radiation therapy have favorable outcomes, and this rate is more than 85% in the modern era. Conclusions Conservative surgery and radiation therapy results in long-term disease control in pediatric patients with craniopharyngioma that is comparable to results obtained with radical surgery alone. However, children with craniopharyngioma remain vulnerable to late treatment failures and side effects from radiation therapy, including endocrinopathies, vasculopathies, and secondary tumors, which may be detrimental to the quality of life. Long-term follow-up beyond 5–10 years is necessary to assess tumor control relative to functional outcomes.

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