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Unformatted text preview: Pediatric Neck Masses Pediatric Neck Masses 1 Mark Domanski, M.D. Michael Underbrink, M.D. Dept. of Otolaryngology University of Texas Medical Branch, Galveston October 31st, 2007 Total % of total 55% 18% 16% 10% 8% 2% Congeital lesions Branchial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele 244 78 73 43 34 10 2 2 1 1 Inflammatory lesions Reactive lympadenopathy Undetermined etiology Sinus histiocytosis Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lympadenitis Sialadenitis 118 27% 71 16% 66 15% 5 1% 32 7% 20 4% 6 1% 2 2 10 2% 5 1% Torsiglieri et al., 19882 Torsiglieri et al., 1988 Non-infammatory benign lesions Inclusion cyst Fibromatosis Keliod 23 5% 13 9 1 3% 2% Benign neoplasms Neurofibroma Lipoma Lipoblastoma Paraganglioma Goiter Benign mixed tumor Osteoblastoma 12 3 3 2 1 1 1 1 3% 1% 1% Malignant neoplasms Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma Total 48 34 23 11 6 2 2 1 1 1 1 445 11% 8% 5% 2% 1% Torsiglieri et al., 19882 Torsiglieri et al., 1988 Malignant neoplasms, 11% Benign neoplasms 3% Non-infammatory benign lesions 5% Congenital lesions 54% Inflammatory lesions 27% N= 445 Congenital Inflammatory H&P Benign Initial Evaluation Initial Evaluation Age Onset Rapidity of growth Fluctuation in size Pain Infection Trauma Travel Exposure PE Size Multiplicity Laterality Consistency Color Mobility Tenderness Fluctuation Malignant Location, Location, Location! Location, Location, Location! Moir. 20048 Age of Distrubtion Age of Distrubtion Range Average (years) Brachial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma 6m – 16 y 9 m – 17 y 9 m – 15 y 9m – 15 y 1 day – 15 y 3.6 6.1 3.7 3.6 5.6 Reactive lymphadenopathy Graunlomatous disease Suppurative lymphadenitis Sialadenitis 3 m – 18 y 8.0 y 1 y – 14 y 6.0 y 4 m – 15 y 7.3 y 11 y – 13 y 11.2 y Inclusion cyst Fibriomatosis 3 y – 12 y 1 m – 10 y 4.4 y 3.1 y Lymphoma Thyroid Carcinoma 4 y – 21 y 8 y – 17 y 11.7 y 12.3 y Others 2 weeks – 18 y 4.6 y y y y y y Torsiglieri et al., 19882 Torsiglieri et al., 1988 Likely Etiology Determines Likely Etiology Determines Direction of Testing X­ray U/S CT MRI FNA Surgical Biopsy Tissue Culture CXR Labs PPD Gram stain Culture Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms 1. Congenital Lesions 1. Congenital Lesions Branchial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele 78 18% 73 16% 43 10% 34 8% 10 2% 2 2 1 1 Embryology Embryology Ectoderm, mesoderm, endoderm Incomplete closure may result in branchial cleft anomalies Moir. 20048 Development of First Four Arches Each arch layer gives rise to: •nerve (ectoderm) •artery, muscle and cartilage (mesoderm) •glands (endoderm). Nicollas. 20003 Branchial Cleft Anomalies Cyst Sinus Fistula Schroeder. 20074 Distribution of neck malformations as cysts, fistulas, or sinuses per Nicollas et. al. (n=191) (Sinus) Total 139 5 47 191 Nicollas. 20003 Moir. 20048 Imagining in Branchial Cleft Cysts Imagining in Branchial Cleft Cysts MRI More reliably confirms cystic nature More precisely defines lesion Better to delineate glandular tissue ie fat planes CT U/S Adequate for most lesions Cost, availability cystic vs noncystic does not evaluate extent Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children. Branstetter, 20069 1st Branchial Cleft Cyst, Type II Type I Ectodermal duplication of EAC Near external auditory canal Usually inferior and posterior to tragus Type II Associated with submandibular gland Branstetter, 20069 Lymphangioma Type 1 First Branchial Cleft Cyst Type 1 First Branchial Cleft Cyst mass Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children. auricle parotid Branstetter, 20069 Branchial Branchial Cyst Noncalcified mass CT shows lesion under SCM Malik et al, 20026 2nd Brachial Cleft Cyst T2 MRI Posterior to R submandibular gland Thickened walls suggest prior hemorrhage or infection Gujar and Mukherji 20045 Anterior to carotid bifurcation Schroeder et al, 20074 Under the anterior SCM Schroeder et al, 20074 Exiting skin medial to lateral border of SCM Schroeder et al, 20074 Left 2nd BA Fistula Left 2 Anterior to carotid bifurcation Under the anterior SCM Exiting skin medial to lateral border of SCM Schroeder et al, 20074 Moir. 20048 Moir. 20048 Moir. 20048 2nd BA cyst with sinus tract extending into the pharynx above the carotid bifurcation Watch the hypoglossal! Schroeder et al, 20074 Preauricular Sinus Preauricular Sinus Not related to 1st branchial cleft anomalies Active infection during excision increases chance of recurrance Moir. 20048 Thyroglossal Duct Cyst Thyroglossal Duct Cyst persistent tract from the descent of the thyroid from the foramen cecum epithelial lining composed of either squamous or respiratory epithelium confirm normal thyroid tissue Learning Radiology.com 200711 Thyroglossal Duct Cyst Thyroglossal Duct Cyst CT w/ Contrast Embedded in the strap muscles Extends deep to involve the pre­ epiglottic space Gujar and Mukherji 20045 Moir. 20048 Moir. 20048 Dermoid Cysts Dermoid Cysts Ectoderm and mesoderm 7% of dermoid cysts occur in head and neck Thought to be of congenital inclusion type mean diameter = 1.2 cm (0.6­3.3) Treatment: complete excision Pryor et al 200512 Dermoid Cysts – Cranial Theory Dermoid Cysts – Cranial Theory Grunwald in 1910 As neuroectodermal tract recedes, demal attachements follow its course and can form a sinus or cyst Beware of possible intracranial involvement Pryor et al 200512 in head & neck, n = 59 •Orbit is the most common site for dermoids in the head and neck (61%) •Direct excision is sufficient for neck dermoids, more extensive approaches (craniotomy, mastoidectomy) are needed for other sites Diff dx: in midline of neck: thyroglossal duct cyst Pryor et al 200512 Dermoid Cysts Dermoid Cysts H & E CT of dermoid cyst Pryor et al 200512 Teratoma Teratoma H&N account for ~2% of teratomas Newborn – 2.5 yr at presentation All 3 germinal layers present Mostly benign lesions amenable to curative excision Wakhlu A et al 200013 Teratoma Teratoma • Prognosis good if no respiratory compromise • Usually well differentiated and recurrence is uncommon • Antenatal diagnosis is routine in developed world Wakhlu A et al 200013 Teratoma Teratoma • Proximity to vital structures makes surgery technically demanding. • Evaluate post op thyroid and parathyroid function. Wakhlu A et al 200013 Teratoma – 3 germ layers Teratoma – 3 germ layers Arise from pluripotent cells and ectopic embryogenic non­germ cells Wakhlu A et al 200013 Teratoma – 3 germ layers Teratoma – 3 germ layers Wakhlu A et al 200013 Teratoma – 3 germ layers Teratoma – 3 germ layers Wakhlu A et al 200013 Hypopharyngeal Teratoma Hypopharyngeal Teratoma calcified calcification and fat Malik et al, 20026 Teratoma Teratoma T1 MRI Calcified Fatty Gujar and Mukherji 20045 Lymphangioma Lymphangioma Benign, multiloculated, soft Posterior neck triangle predominance Multi­septated, insinuating lesions Infiltrate and cross tissue planes Most occur by 2 yrs of age Incidence: 1 in 6,000 to 16,000 births Head and and Neck Surgery, 200615 Burezq 200614 Lymphatic Vascular malformation Lymphatic Vascular malformation T1 MRI High signal represents proteinaceous fluid Crosses tissue planes Gujar and Mukherji 20045 Centrifugal vs Centripetal Centrifugal vs Centripetal Centrifugal theory the lymphatic system develops as mesenchymal spaces that later coalesce into a system of vessels that eventually join the venous system. Centripetal theory jugular and posterior lymphatics form as outgrowths of endothelium from veins into the surrounding mesenchyme. Burezq 200614 Classification Classification Size: Microcystic: capillary lymphangiomas lesions are less than 1 cm in diameter Macrocystic: cystic hygromas cysts are larger than 1 cm Cystic hygromas #1 type of lymphangioma Gross et al, 200616 Cystic Hygroma Cystic Hygroma Noncalcified Septated on U/S Malik et al, 20026 Cystic Composition Cystic Composition 5­year­old boy with lymphangioma L parotid & parapharyngeal space mixed macro­ andmicrocystic type Treated by surgical resection Gross et al, 200616 Lymphangioma Type 1 First Branchial Cleft Cyst Type 1 First Branchial Cleft Cyst mass Both MRI and CT have difficulty distinguishing branchial cleft cyst from lymphangioma in children. auricle parotid Branstetter, 20069 Burezq et al, 2006 Burezq et al, 2006 (expert opinion) 1. Error in establishing a communication between the lymphatic and venous system Cystic hygroma 2. Error in morphogenesis of lymphatic system: this includes other types of lymphatic malformations microcystic, macrocystic and mixed lymphatic lesions Burezq 200614 Management ­ Controversial Management ­ Controversial Spontaneous resolution? Formation of new lymphatic channels? Serial aspiration? Sclerosant Agents? OK­432 (lyophilizied mixture of low­virulence group A Sterp pyogens Surgical Excision? Is the surgical risk out weigh the benefit in a benign lesion Burezq 200614 Success with Serial Aspirations Success with Serial Aspirations Burezq 200614 Success with OK­432 Success with OK­432 Supraclavicular macrocystic lymphangioma Gross et al, 200616 Hemangioma Hemangioma Less than 1/3 present at birth Usually seen in 1st few months of life and enlarge progressively 90% cases involutes spontaneously Sclerosing agents controversial Glut­1 erythrocyte­type glucose transporter found only in microvascular endothelia of blood–tissue barriers such as in the central nervous system, retina, placenta, ciliary muscle, and endoneurium of peripheral nerves Hemangiomas stain consistently for Glut­1, in all stages of development and involution whereas vascular malformations did not MacArther, 200618 Mo et al, 200617 Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms 2. Inflammatory Lesions 2. Inflammatory Lesions Reactive lympadenopathy Undetermined etiology Sinus histiocytosis Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lympadenitis Sialadenitis 71 16% 66 15% 5 1% 32 7% 20 4% 6 1% 2 2 10 2% 5 1% When does cervical When does cervical lymphadenopathy require FNA? Benign reactive lymph node may persist for weeks to months Lymphoma can present the same way Rapkiewicz et al 200721 To FNA or not to FNA? To FNA or not to FNA? Reactive lymphadenopathy the most likely etiology of pediatric neck masses Diagnostic dilema: a mass that does not resolve after initial treatment Rapkiewicz et al 200721 FNA ancillary studies FNA ancillary studies Gram stain, culture Acid fast stain Imunocytochemistry Cytogenetics Rapkiewicz et al 200721 Limitations to FNA Limitations to FNA A lesion may not be homogenous FNA samples only part of the mass May miss the true lesion Unable to appreciate histological architecture Rapkiewicz et al 200721 Time to contemplate open biopsy Time to contemplate open biopsy Enlarging mass Poor response to medical treatment Suspicious clinical course Unusual image findings Systemic symptoms Rapkiewicz et al 200721 Case – F.R. Case – F.R. 8 y/o female, hx + PPD several yrs prior Presents with R cervical adenopathy FNA suggests granuloma Repeat FNA ­> same result AFB stain and cultures negative Clarithromycin and ethambutol started Rapkiewicz et al 200721 Case – F.R. Case – F.R. Adenopathy and pain increased Third FNA non­diagnostic CT shows bulky homogenous lymphadenopathy of R upper spinal accessory and upper jugular chains. Open biopsy displayed Hodgkin's lymphoma. Rapkiewicz et al 200721 Reactive Lymphadenopathy Reactive Lymphadenopathy 3­year­old child Multiple hypoechoic lesions variable shape and sizes consistent with reactive lymph nodes Malik et al, 20026 Enlarged Lymph Node Enlarged Lymph Node Nonspecific Cause: cryptococcal adenitis Yeastlike fungus Gujar and Mukherji 20045 Atypical mycobacteria: ex: Atypical mycobacteria: ex: cryptococcus Saprobe in nature worldwide distribution Found in soil Portal of entry is lung Atypical mycobacteria: ex: Atypical mycobacteria: ex: cryptococcus Associated w/ AIDS organ transplantation Lymphoreticular diseases ½ pts lack apprarent predisposing factors Bartonella henselae Bartonella henselae Hypoechoic masses with irregular rim of isoechoic tissue Biopsy: Cat Scratch Disease Malik et al, 20026 Bartonella henselae Bartonella henselae In heart valve Gram – coccobacillus 2­ 14 day incubation Dx: requires prolonged incubation (2 + weeks) Rx: erythromycin 1­4 m (unclear efficacy) Normally benign course Malik et al, 20026 Peritonsillar Abcess Peritonsillar Abcess Soft tissue density in submental space Malik et al, 20026 Retropharyngeal Retropharyngeal Abscess Widening of prevertebral space Malik et al, 20026 Retropharyngeal Space Retropharyngeal Space Abscess Gujar and Mukherji 20045 Peritonsillar Retropharyngeal Malik et al, 20026 Sppurative Cervical Adenitis Sppurative Cervical Adenitis hypoechoic Malik et al, 20026 Thyroid Abscess Thyroid Abscess Malik et al, 20026 Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms 3. Non­inflammatory Benign 3. Non­inflammatory Benign Lesions Inclusion cyst Fibromatosis Keloid 13 9 1 3% 2% Inclusion Cyst Inclusion Cyst Acquired dermoid cysts result from a part of the skin being traumatically implanted in the deeper layers after ectopic formation of a dermal cyst lined with squamous epithelium. Congenital inclusion dermoid cysts form along the lines of embryologic fusion and contain both dermal and epidermal derivatives. Dermoid cysts of the head and neck are thought to be the congenital inclusion type. Pryor et al 200512 Inclusion Cyst Inclusion Cyst many cysts originate from the infundibular portion of the hair follicle, and the more general term, epidermoid cyst, is favored Becker et a, 200519 Inclusion Cyst Inclusion Cyst Discharge of a foul­smelling cheeselike material is a common complaint. Less frequently, the cysts can become inflamed or infected, resulting in pain and tenderness. In the uncommon event of malignancy, rapid growth, friability, and bleeding have been reported Injuries, especially of the crushing type, such as the slamming of a car door on a finger, are frequently reported in association with subungual or terminal phalanx epidermoid cysts. Theoretically, any surgical procedure may result in epidermoid cysts, and it is surprising that they are not a more common occurrence Becker et al, 200519 Epidermal Inclusion Cyst Epidermal Inclusion Cyst Cyst containing keratinous material true epidermis with a granular layer and adjacent laminated keratinous material Becker et al, 200519 Torticollis Torticollis Fibromatosis Fibromatosis Colli SCM Isoechoic mass CT shows isodense mass R side Note normal SCM on L side Malik et al, 20026 Fibromatosis Colli ­ FNA Fibromatosis Colli ­ FNA Paucicellular specimen Bland spindle cell cytology r/o nodular fascitis and fibrosarcoma Rapkiewicz et al 200721 Fibromatosis Colli Fibromatosis Colli r/o nodular fascitis and fibrosarcoma Roy, 200720 Moir. 20042 Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms 4. Benign Neoplasms 4. Benign Neoplasms Neurofibroma Lipoma Lipoblastoma Paraganglioma Goiter Benign mixed tumor Osteoblastoma 3 3 2 1 1 1 1 1% 1% Neurofibroma Neurofibroma solitary lesion vs part of the generalized syndrome of neurofibromatosis NF­1, aka von Recklinghausen disease NF­2 Believed to arise from Schwann cell but origin uncertain Neurofibroma Neurofibroma solitary lesion vs part of the generalized syndrome of neurofibromatosis NF­1, aka von Recklinghausen disease NF­2 Believed to arise from Schwann cell but origin uncertain Neurofibroma Neurofibroma T2 MRI Central low T2 signal is characteristic of neurofibromas Gujar and Mukherji 20045 Lipoma Lipoma Lipoblastoma Lipoblastoma FNA Rare benign mesynchymal tumor of embryonal fat May clinically and radiologically mimic a hemangioma Collections of lipoblasts – multivuolated w/ round nuclei Lipoblastoma Lipoblastoma Surgical specimen Resembles embryological adipose tissue Lipoma Lipoblastoma Lipoma Lipoblastoma Neonatal Neonatal Goiter CT shows large peripheral rim enhancing, low attenuation mass 1: 4000 live births Female 2x = Male predominance Delayed ossification at bone ends Rovet et al, 200310 Malik et al, 20026 Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms Pediatric Neck Masses Pediatric Neck Masses 1. Congenital lesions 2. Inflammatory lesions 3. Non­inflammatory benign lesions 4. Benign neoplasms 5. Malignant neoplasms 5. Malignant Neoplasms 5. Malignant Neoplasms Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma 34 23 11 6 2 2 1 1 1 1 8% 5% 2% 1% Lymphoma Lymphoma Third most common pediatric cancer Incidence: 11­20 per million children Geographical variance – 50 % of childhood cancers in equatorial Africa Due to high incidence of Burkitt’s lymphoma Male predominance 2.5:1 Beware the supraclavicular mass! Beware the supraclavicular mass! Turkington et al 2005 22 35% of patients with H&N lymphoma present with a supraclavicular mass 35% of pts with suprclavicular masses had lymphoma Torsiglieri et al., 19882 Roh et al 20077 Neuroblastoma Neuroblastoma Noncontrast T1 MRI Mass (arrow) lateral to carotid artery (arrowhead). Gujar and Mukherji 20045 Rhabdomyosarcoma ­ CT Rhabdomyosarcoma ­ CT Ill defined enhancing soft tissue density areas of necrosis Malik et al, 20026 Rhabdomyosarcoma Rhabdomyosarcoma of the Masticator Space Non­contrast T1 – intermediate signal T2 – increased signal Gujar and Mukherji 20045 Torsiglieri et al., 19882 Torsiglieri et al., 1988 Malignant neoplasms, 11% Benign neoplasms 3% Non-infammatory benign lesions 5% Congenital lesions 54% Inflammatory lesions 27% N= 445 Total % of total 55% 18% 16% 10% 8% 2% Congeital lesions Branchial cleft cyst Thyroglossal duct cyst Dermoid cyst Lymphangioma Hemangioma Teratoma Bronchogenic cyst Thymic cyst Myelomeningocele 244 78 73 43 34 10 2 2 1 1 Inflammatory lesions Reactive lympadenopathy Undetermined etiology Sinus histiocytosis Granulomatous disease Atypical mycobacteria Cat scratch disease Toxoplasmosis Sarcoid Suppurative lympadenitis Sialadenitis 118 27% 71 16% 66 15% 5 1% 32 7% 20 4% 6 1% 2 2 10 2% 5 1% Torsiglieri et al., 19882 Torsiglieri et al., 1988 Non-infammatory benign lesions Inclusion cyst Fibromatosis Keliod 23 5% 13 9 1 3% 2% Benign neoplasms Neurofibroma Lipoma Lipoblastoma Paraganglioma Goiter Benign mixed tumor Osteoblastoma 12 3 3 2 1 1 1 1 3% 1% 1% Malignant neoplasms Lymphoma Hodgkin's Non-Hodkin's Thyroid Carcinoma Rhabdomyosarcoma Neuroblastoma Fibrous histiocytoma Acinic cell carcinoma Histiocytosis X Chloroma Total 48 34 23 11 6 2 2 1 1 1 1 445 11% 8% 5% 2% 1% Conclusions Conclusions Initial evaluation (H&P) Congenital, infectious, benign, malignant Beware of tuberculosis, cat scratch disease, atypical infections Beware of systemic symptoms Beware the supraclavicular mass Consider FNA or biopsy inthe mass that does not resolve with treatment. 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