Isolated cerebellar Rosai-Dorfman granuloma mimicking Lhermitte-Duclos disease

2009 ◽  
Vol 4 (2) ◽  
pp. 118-120 ◽  
Author(s):  
James M. Johnston ◽  
David D. Limbrick ◽  
Wilson Z. Ray ◽  
Stephanie Brown ◽  
Joshua Shimony ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Histological Examination ◽  
Pediatric Population ◽  
Cerebellar Lesion ◽  
Presumptive Diagnosis ◽  
Suboccipital Craniotomy ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

scholarly journals Paranasal Mass in a Healthy Male Toddler

2021 ◽  
pp. 014556132110079
Author(s):  
Melonie Anne Phillips ◽  
Meredith Lind ◽  
Gerd McGwire ◽  
Diana Rodriguez ◽  
Suzanna Logan
Keyword(s):  
Differential Diagnosis ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Age Group ◽  
Significant Morbidity ◽  
Healthy Male ◽  
Neck Tumors ◽  
Complete Surgical Resection ◽  
Maxilla And Mandible ◽  
Benign Mass

Head and neck tumors are rare in pediatric patients but should be kept in the differential when a patient presents with a new swelling or mass. One of these tumors is a myxoma, which is an insidiously growing, benign mass originating from the mesenchyme. They most commonly arise in the myocardium but can also develop in facial structures, particularly in the maxilla and mandible. When arising in facial structures, ocular, respiratory, and digestive systems can be affected based on local invasion. Complete surgical resection is curative but can lead to significant morbidity as well. Here, we present a case of a 15-month-old toddler presenting with a paranasal mass, which was ultimately diagnosed as a maxillary myxoma. This tumor is very rare in the pediatric population, especially in the toddler age-group, reminding clinicians to broaden the differential diagnosis when a patient’s course is atypical.

Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan
Keyword(s):  
Differential Diagnosis ◽  
Pituitary Gland ◽  
Plasma Cells ◽  
S100 Protein ◽  
Clinical Context ◽  
Cutaneous Involvement ◽  
Eosinophilic Infiltrate ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Skin Biopsies

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

scholarly journals Pulsatile Midline Solitary Plasmacytoma in the Frontal Head Region-A Case Report”

2016 ◽  
Vol 32 (1) ◽  
pp. 39-42
Author(s):  
Md Atikur Rahman ◽  
Aklaque Hossain Khan ◽  
Kanak Kanti Barua
Keyword(s):  
Multiple Myeloma ◽  
Differential Diagnosis ◽  
Case Report ◽  
Histological Examination ◽  
Plasma Cells ◽  
Extramedullary Plasmacytoma ◽  
Solitary Plasmacytoma ◽  
Rare Entity ◽  
Head Region ◽  
Malignant Plasma Cell

Primary craniocerebral plasmacytomas are uncommon and represent only 0.7 % of all plasmacytomas. In this case solitary plasmacytoma in the midline frontal head region of the skull and discuss the clinical features and prognosis of this tumor. Plasmacytoma can present as multiple myeloma, solitary plasmacytoma of the bone or extramedullary plasmacytoma. Solitary plasmacytoma is a rare entity that composes of malignant plasma cells and involves the bone to form only one or two lesions without evidence of disease dissemination. It accounts for only 4% of malignant plasma cell tumors. 50 years old male was suffering from plasmacytoma in the frontal head region in our case which is pulsatile. On images showed multiple differential diagnosis but after operation histological examination revealed plasmacytoma. Bangladesh Journal of Neuroscience 2016; Vol. 32 (1): 39-42

scholarly journals Intradural Lipoma at the Craniocervical Junction Presenting with Progressing Hemiparesis: A Case Report

2019 ◽  
Vol 11 (2) ◽  
pp. 183-188
Author(s):  
Takuro Inoue ◽  
Hisao Hirai ◽  
Ayako Shima ◽  
Fumio Suzuki ◽  
Masayuki Matsuda ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Pediatric Population ◽  
Adult Population ◽  
Spinal Dysraphism ◽  
Craniocervical Junction ◽  
Suboccipital Craniotomy ◽  
Benign Nature ◽  
Intradural Lipoma ◽  
Radiological Studies ◽  
Dorsal Medulla

Intradural spinal lipomas are rare in an adult population. They are mostly asymptomatic and usually associated with spinal dysraphism in a pediatric population. We report a rare case of spinal lipoma without dysraphism and with progressing hemiparesis. A 60-year-old woman had incidental lipoma at the craniocervical junction observed for more than 5 years. Recently, she developed right-sided hemiparesis and sensory disturbance. Radiological studies revealed a large lipoma compressing the dorsal medulla and C1–C2 spinal cord. Standard midline suboccipital craniotomy and C1 laminectomy were performed, and the lipoma was removed subtotally. The lipoma showed severe adhesion to the dorsal medulla and C1 spinal cord; therefore, the excision was limited as internal debulking. Her neurological deficit subsided within 6 months after the decompressive surgery. Considering the benign nature of lipoma, internal decompression is a reasonable management for this lesion.

scholarly journals Localized Eruptive Blue Nevi after Herpes Zoster

2016 ◽  
Vol 8 (2) ◽  
pp. 118-123 ◽  
Author(s):  
Fany Colson ◽  
Jorge E. Arrese ◽  
Arjen F. Nikkels
Keyword(s):  
Differential Diagnosis ◽  
Herpes Zoster ◽  
Histological Examination ◽  
Excisional Biopsy ◽  
Left Shoulder ◽  
Oral Acyclovir ◽  
Blue Nevus ◽  
Short Period ◽  
Previous Trauma ◽  
Dark Blue

A 52-year-old White man presented with a dozen small, well-restricted, punctiform, asymptomatic, blue-gray macules on the left shoulder. A few months earlier, he had been treated with oral acyclovir for herpes zoster (HZ) affecting the left C7–C8 dermatomes. All the blue macules appeared over a short period of time and then remained stable. The patient had not experienced any previous trauma or had tattooing in this anatomical region. The clinical diagnosis suggested blue nevi. Dermatoscopy revealed small, well-limited, dark-blue, compact, homogeneous areas evoking dermal blue nevi. An excisional biopsy was performed and the histological examination confirmed a blue nevus. As far as we are aware of, this is the first report of eruptive blue nevi following HZ, and it should be included in the differential diagnosis of zosteriform dermatoses responding to an isotopic pathway. In addition, a brief review concerning eruptive nevi is presented.

scholarly journals Pilomatrixoma of eyelid in a middle aged

1970 ◽  
Vol 3 (1) ◽  
pp. 88-90 ◽  
Author(s):  
N Passi ◽  
U Chawla ◽  
A Joyti ◽  
AK Khurana
Keyword(s):  
Differential Diagnosis ◽  
Histopathological Examination ◽  
Meibomian Gland ◽  
Excision Biopsy ◽  
Middle Aged ◽  
Lateral Aspect ◽  
Presumptive Diagnosis ◽  
Skin Nodule ◽  
Upper Lid ◽  
Gland Carcinoma

Background: Pilomatrixoma , also known as Malherbes’s calcifying epithelioma, known to occur in children, is not common especially in middle aged and those below 10 years of age . Pilomatrixoma presents as a firm skin nodule on eye lid or eye brow, so should be considered in the differential diagnosis of such lesions involving eyelids. Case: A 45-year-old male presented with a swelling in the left upper lid for the last 4 months. There was firm to hard non tender nodule measuring 1.5 cm. x 1.5cm in size on the lateral aspect of the left upper eye lid , involving the lid margin with variegated appearance of overlying skin with a few ulcerated areas on the surface. A presumptive diagnosis of meibomian gland carcinoma was made and an excision biopsy with primary lid repair was carried out. Histopathological examination confirmed the diagnosis of pilomatrixoma. Conclusion: The present case is reported to sensitize the ophthalmologists to think beyond chalazion and meibomian gland carcinoma for a firm, nodular, non ulcerated, painless lid swelling. Key words: Pilomatrixoma; Malherbes's calcifying epithelioma; eyelid nodule  DOI: 10.3126/nepjoph.v3i1.4286Nepal J Ophthalmol 2011;3(5):88-90

scholarly journals Late presentation of hyperandrogenism in pregnancy: clinical features and differential diagnosis

2013 ◽  
Vol 2013 ◽  
Author(s):  
Gautam Das ◽  
Vinay S Eligar ◽  
Jyothish Govindan ◽  
D Aled Rees
Keyword(s):  
Differential Diagnosis ◽  
Clinical Features ◽  
Post Partum ◽  
Late Presentation ◽  
List Type ◽  
Presumptive Diagnosis ◽  
Second Trimester Pregnancy ◽  
Ovarian Pathology ◽  
Pregnant State ◽  
In Pregnancy

Summary Background: Hyperandrogenic states in pregnancy are rare but arise most commonly due to new-onset ovarian pathology in pregnancy. We describe the case of a young woman who presented in the latter half of her pregnancy with features of hyperandrogenism. We review the biochemical and imaging findings and discuss the differential diagnosis. Case presentation: A 26-year-old woman presented in the later part of her pregnancy with widespread hirsutism. Biochemical testing confirmed hyperandrogenism (testosterone, 13.7 nmol/l and second-trimester pregnancy range, 0.9–4.9 nmol/l), although she had no history of menstrual disturbance, hirsutism or acne prior to conception. Radiological evaluation (ultrasound and magnetic resonance imaging) revealed multiple cystic lesions in both ovaries, leading to a presumptive diagnosis of hyperreactio luteinalis (HL). The implications of maternal hyperandrogenism on foetal virilisation were considered and the patient was counselled appropriately. She delivered a healthy baby boy uneventfully. Androgen levels, hirsutism and acne normalised within a few weeks of delivery. Conclusion: HL can occur at any stage of pregnancy and is an important differential diagnosis in pregnant patients with features of androgen excess. Most cases regress spontaneously after delivery and major interventions are usually not needed. Learning points Hyperandrogenism in pregnancy is rare. Clinical features are similar to the non-pregnant state in the mother but virilisation in the foetus can have profound consequences. HL and pregnancy luteoma are the most common ovarian pathologies leading to hyperandrogenism in pregnancy. Spontaneous regression occurs in the post-partum period in the vast majority of cases and surgery is only required for local complications.

scholarly journals Unilateral Hemiparesis with Thoracic Epidural in an Adolescent

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Rosalie F. Tassone ◽  
Christian Seefelder ◽  
Navil F. Sethna
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Epidural Anesthesia ◽  
Pediatric Population ◽  
Motor Blockade ◽  
Neural Blockade ◽  
Epidural Catheter Placement ◽  
Thoracic Epidural ◽  
Thoracic Epidural Catheter ◽  
Correct Location

Objective. Unilateral sensory and motor blockade is known to occur with epidural anesthesia but is rarely reported in children. The differential diagnosis should include the presence of a midline epidural septum.Case Report. We describe a case of a 16-year-old adolescent who developed repeated complete unilateral extensive epidural sensory and motor blockade with Horner’s syndrome after thoracic epidural catheter placement. This unusual presentation of complete hemibody neural blockade has not been reported in the pediatric population. Maneuvers to improve contralateral uniform neural blockade were unsuccessful. An epidurogram was performed to ascertain the correct location of the catheter within the epidural space and presence of sagittal compartmentalization.Conclusion. This case report highlights a less frequently reported reason for unilateral sensory and motor blockade with epidural anesthesia in children. The presence of a midline epidural septum should be considered in the differential diagnosis of unilateral epidural blockade.

scholarly journals Hereditary Neuropathy with Liability to Pressure Palsy: A Recurrent and Bilateral Foot Drop Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Filipa Flor-de-Lima ◽  
Liliana Macedo ◽  
Ricardo Taipa ◽  
Manuel Melo-Pires ◽  
Maria Lurdes Rodrigues
Keyword(s):  
Histological Examination ◽  
Pediatric Population ◽  
Sensory Nerve ◽  
Foot Drop ◽  
First Episode ◽  
Hereditary Neuropathy ◽  
Minor Trauma ◽  
Motor Rehabilitation ◽  
Sensorimotor Polyneuropathy ◽  
Pressure Palsy

Hereditary neuropathy with liability to pressure palsy is characterized by acute, painless, recurrent mononeuropathies secondary to minor trauma or compression. A 16-year-old boy had the first episode of right foot drop after minor motorcycle accident. Electromyography revealed conduction block and slowing velocity conduction of the right deep peroneal nerve at the fibular head. After motor rehabilitation, he fully recovered. Six months later he had the second episode of foot drop in the opposite site after prolonged squatting position. Electromyography revealed sensorimotor polyneuropathy of left peroneal, sural, posterior tibial, and deep peroneal nerves and also of ulnar, radial, and median nerves of both upper limbs. Histological examination revealed sensory nerve demyelination and focal thickenings of myelin fibers. The diagnosis of hereditary neuropathy with liability to pressure palsy was confirmed by PMP22 deletion of chromosome 17p11.2. He started motor rehabilitation and avoidance of stressing factors with progressive recovery. After one-year followup, he was completely asymptomatic. Recurrent bilateral foot drop history, “sausage-like” swellings of myelin in histological examination, and the results of electromyography led the authors to consider the diagnosis despite negative family history. The authors highlight this rare disease in pediatric population and the importance of high index of clinical suspicion for its diagnosis.

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