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23 Vascular Malformations andTumors

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Kaposiform Hemangioendothelioma (KH) andTufted Angiomas (TA)

• KH is benign vascular tumors appearing as violaceous cutaneous nodules that extend into deep tissues

• TA are more localized than KH and may not involve the skin

• Unlike infantile hemangioma, they do not spontaneously involute

• Have an endothelial and lymphatic component

• 42% present with Kasabach–Merrit phenomenon (see below)

• Treatment is with surgery ± chemotherapy

Kasabach–Merritt Phenomenon

• Severe thrombocytopenia that can occur in Kaposiform hemangioendothelioma or tufted angioma

• Treated with chemotherapy to decrease the risk of bleeding and may require transfusion to treat active bleeding

PHACES Syndrome

• Hemangioma associated with the following:

– Posterior fossa malformation – Hemangioma – Arterial abnormality and coarctation of the aorta – Cardiac defects – Eye abnormality – Sternal clefting

Symptoms

• Developmental delay, seizure, congenital stroke

Work Up

• Ophthalmology, cardiac, and neurologic investigation

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B. M. Laitman et al.

Osler–Weber–Rendu Syndrome

• Aka Hereditary hemorrhagic telangiectasia (HHT)

• Autosomal dominant syndrome affecting blood vessels throughout body

Presentation

• Ectatic vessels of skin, mucous membranes, and viscera

• Epistaxis usually presenting symptom at puberty/early adulthood

• Skin telangiectasia can be punctate, linear, or spider like

• Mucous membranes of oral cavity, naso/oropharynx, and GI tract

• Associated with pulmonary, cerebral, and hepatic arteriovenous malformations

Diagnosis

• Hemorrhagic episodes (nasal cavity/nasopharynx, GI)

• Vascular ectasia

• Family history

Investigation

• Genetic testing available

• Must rule out cerebral and pulmonary AVM with imaging in children with family history

Treatment

• Supportive

• Electrocoagulation, photocoagulation (pulse-dye, Nd:YAG, argon beam)

– Bevacizumab (VEGF inhibitor) to reduce nosebleeds

Maffucci Syndrome

• Cavernous hemangiomas

• Chondrosarcoma (25% of cases)

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• Dyschondroplasia

• Visceral vascular lesions

Sturge–Weber Syndrome (SWS)

• Capillary malformation involving eye, skin, and leptomeninges

• Nevus Flammeus (port wine stain) is a cutaneous facial venous dilation, hallmark of SWS, and present at birth

• Seizure in up to 85% of patients, intellectual disability in 60%

• Requires ophthalmology and neurology workup

Further Reading

1. Alster TS, Railan D. Laser treatment of vascular birthmarks. J Craniofac Surg. 2006;17(4):720–3.

2. Antman K, Chang Y.Kaposi’s sarcoma. N Engl J Med. 2000;342(14):1027–38.

3. Buckmiller LM, etal. Propranolol for infantile hemangiomas: early experience at a tertiary vascular anomalies center. Laryngoscope. 2010;120(4):676–81.

4. Chandler JR, etal. Nasopharyngeal angiobromas: staging and management. Ann Otol Rhinol Laryngol. 1984;93(4 Pt 1):322–9.

5. Comi AM.Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-weber syndrome. Neurologist. 2011;17(4):179–84.

6. de Serres LM, Sie KC, Richardson MA.Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg. 1995;121(5):577–82.

7. Dompmartin A, Vikkula M, Boon LM.Venous malformation: update on aetiopathogenesis, diagnosis and management. Phlebology. 2010;25(5):224–35.

8. Duval M, Hwang E, Kilty SJ. Systematic review of treatment and prognosis of sinonasal hemangiopericytoma. Head Neck. 2013;35(8):1205–10.

9. Enjolras O, etal. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr. 1997;130(4):631–40.

10. Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol. 1996;132(3):307–11.

11. Kim MG, etal. The therapeutic effect of OK-432 (picibanil) sclerotherapy for benign neck cysts. Laryngoscope. 2008;118(12):2177–81.

12. Kohout MP, etal. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg. 1998;102(3):643–54.

13. Mendenhall WM, etal. Cutaneous angiosarcoma. Am J Clin Oncol. 2006;29(5):524–8.

14. Mendenhall WM, etal. Head and neck paragangliomas. Head Neck. 2011;33(10):1530–4.

15. Nicolai P, etal. Endoscopic surgery for juvenile angiobroma: when and how. Laryngoscope. 2003;113(5):775–82.

16. Oldring D, Fisch U. Glomus tumors of the temporal region: surgical therapy. Am J Otol. 1979;1(1):7–18.

17. Pasini B, Stratakis CA.SDH mutations in tumorigenesis and inherited endocrine tumours: lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med. 2009;266(1):19–42.

18. Shovlin CL, etal. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Oslerweber syndrome). Am J Med Genet. 2000;91(1):66–7.

Chapter 24

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Cutaneous andTemporal Bone Malignancies

KevinWong, HailunWang, BrettA.Miles, andMohemmedKhan

Pearls

• The most signicant risk factor to cutaneous malignancy is ultraviolet (UV)

radiation

• UVB light (200–320nm) more carcinogenic than UVA light (320–400nm)

• Basal cell carcinoma (BCC) most common cutaneous malignancy

• For melanoma, tumor depth is the most important prognostic factor for stage 1

and 2 disease; however, lymph node involvement is the most important factor for stage 3 disease

• Squamous cell carcinoma (SCC) accounts for 70% of all tumors in the ear canal,

middle ear, and mastoid cavity

• Lateral temporal bone resection with or without auriculectomy, craniotomy,

mandibulectomy, neck dissection, or parotidectomy may be needed for advanced temporal bone malignancies

K. Wong (*) · M. Khan Department of Otolaryngology - Head and Neck Surgery, Icahn School of Medicine at the Mount Sinai Hospital, New York, NY, USA e-mail: Kevin.wong@mountsinai.org; mohemmed.khan@mountsinai.org

H. Wang Department of Otolaryngology, University of Connecticut, Farmington, CT, USA e-mail: hwang@prohealthmd.com

B. A. Miles Otolaryngology Head and Neck Surgery, Oral and Maxillofacial Surgery, Northwell Health System, New Hyde Park, NY, USA e-mail: bmiles4@northwell.edu

https://doi.org/10.1007/978-3-031-26048-3_24

441

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• Adjuvant chemoradiotherapy may serve a role for advanced temporal bone

malignancies

• High-dose interferon-a 2b is the only FDA-approved adjuvant therapy for stage

III melanoma

K. Wong et al.

Anatomy

• Layers of skin (supercial to deep)

– Epidermis

Stratum corneum Stratum granulosum Stratum spinosum Stratum germinativum/basale

– Dermis

Papillary dermis Reticular dermis

– Melanocytes=melanin-producing cells located in stratum basale; responsible

for skin pigmentation

– Basal cells=small, round, epithelial cells found in stratum basale

• Temporal bone

– Four parts: squamous, mastoid, petrous, and tympanic – Middle ear (points of spread)

Anterior: Eustachian tube Medial: round window, oval window Posterior: mastoid cavity Superior: tegmen tympani

– External auditory canal (EAC)

Lateral 1/3: cartilaginous, cerumen glands (modied sebaceous glands) Medial 2/3: bony, epithelium is adherent to periosteum, resistant to radial spread of cancer Huschke foramen: developmental defect in tympanic ring → anterior extension of EAC malignancy to parotid Fissures of Santorini: small dehiscences in anterior cartilage → direct radial EAC tumor extension

• Lymphatic drainage

– Auricle and EAC: periparotid lymph nodes (LN), parotid gland, jugular chain,

LN overlying mastoid

– Medial EAC, middle ear: retropharyngeal nodes, deep jugular nodes

24 Cutaneous andTemporal Bone Malignancies

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Basal Cell Carcinoma

Epidemiology

• Most common skin cancer (65%)

• Worldwide incidence increasing

• Locally destructive but least likely to metastasize (risk 0.1%)

• Average age at diagnosis=64.4years old

Presentation

• Slowly enlarging “pearly” skin lesion, ulceration, papule/nodule within scar

• Local tissue destruction

• Fair skin (Fitzpatrick scale type 1) has 10–20× higher risk

Etiology/Pathogenesis

• Ultraviolet radiation

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– Sunlight, tanning booths, UV light therapy

Latency period 20–50years

– UV radiation→modify nucleic acids → activation of oncogenes or inactiva-

tion of tumor suppressor genes

– Other radiation=X-ray, Grenz-ray

• Gene mutations

– TP53 (tumor protein p53) – Patched (PTCH)=tumor suppressor gene, Gorlin syndrome – Patched homologue 1 (PTCH1) = loss-of-function mutation, sporadic and

familial cases of BCC

– Sonic hedgehog (SHH), smoothened (SMO), Gli = gain-of-function muta-

tions, sporadic and familial cases of BCC

• Immunosuppression

– 10× higher incidence of skin cancer in transplant patients

• Other causes:

– Arsenic ingestion (Fowler solution, water contaminant) – Trauma

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– Xeroderma pigmentosum

Autosomal recessive Photosensitive skin with multiple BCCs and other cutaneous malignancies

– Epidermodysplasia verruciformis (Treeman syndrome)

Autosomal recessive Vulnerability to human papillomavirus leading to multiple scaly macules and increased susceptibility to BCC, SCC, and warts

– Nevoid basal cell carcinoma syndrome (Gorlin syndrome)

Autosomal dominant Defect in PTCH 1 tumor suppressor gene on chromosome 9 leading to multiple BCCs, odontogenic keratocysts, hyperkeratosis of palms and soles, intracranial calcications, brain tumors, and rib anomalies

– Bazex syndrome (paraneoplastic acrokeratosis)

Rare paraneoplastic syndrome characterized by follicular atrophoderma, BCCs, and local anhidrosis

– Rombo syndrome

Autosomal dominant Multiple BCC, trichoepithelioma, hypotrichosis, cyanosis of the hands and feet

K. Wong et al.

– Alcohol consumption

Positive association between excessive alcohol use and incidence of sunburns

Diagnosis

• Shave or punch biopsy

• Dermatoscopy: arborizing telangiectasia, blue-grey globules, blue-grey ovoid

nests, spoke wheel areas

• Histopathology

– Undifferentiated (solid type): basaloid cell proliferation with extension into

papillary dermis, palisading peripheral columnar cells

– Differentiated: keratotic (hairlike structures), cystic (sebaceous, gland-like

structures), adenoid (tubular structures, lacelike pattern)

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• High-risk features:

– Tumor >2mm thickness or Clark level≥IV (see Breslow and Clark below) – Perineural invasion (PNI) – High-risk anatomic area: lip (vermilion and hair-bearing), ears, temple, pre-

and postauricular region, central face, eyelids, nose, lips, chin, mandible – Poorly differentiated or undifferentiated histology – Recurrence

• Types (multiple)

– Nodular: low risk, most common, pearly/translucent, rolled edges, telangiec-

tasia, central depression, ± ulceration – Supercial: low risk, scaly, pink, patch or plaque, more common on trunk/

extremities – Morpheaform: high risk, at or depressed, whitish scar; aggressive, worst

prognosis – Pigmented: similar to nodular type, more pigmented, resembles melanoma or

benign nevus – Fibroepithelioma: raised, rm, pedunculated or sessile, red with smooth surface – High risk: basosquamous, micronodular, mixed inltrative, sclerosing

445

Staging

American Joint Committee onCancer (AJCC) Eighth Edition Staging forNon-melanoma Cutaneous Carcinoma oftheHead andNeck

Primary tumor (T) T Criteria

TX Primary tumor cannot be assessed Tis Carcinoma in situ T1 ≤2cm T2 >2cm and≤4cm T3 >4cm or minor bone erosion or perineural invasion or deep invasion T4 Cortical bone/marrow, skull base, and/or skull base foramen invasion T4a Gross cortical bone/marrow invasion T4b Skull base and/or skull base foramen involvement

Deep invasion dened as invasion beyond subcutaneous fat or>6mm

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Regional lymph node (N) Clinical N (cN) N N criteria

NX Lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Single ipsilateral lymph node ≤3cm and ENE(−) N2a Single ipsilateral lymph node 3-6cm and ENE(−) N2b Multiple ipsilateral lymph nodes <6cm and ENE(−) N2c Bilateral or contralateral lymph node(s) >6cm and ENE(−) N3a Single ipsilateral lymph node >6cm and ENE(−) N3b Any lymph node with ENE(+)

Pathological N (pN) N N criteria

NX Lymph nodes cannot be assessed N0 No regional lymph node metastasis N1 Single ipsilateral lymph node ≤3cm and ENE(−) N2a Single ipsilateral lymph node ≤3cm and ENE(+) OR single ipsilateral lymph node

3–6cm and ENE(−) N2b Multiple ipsilateral lymph nodes <6cm and ENE(−) N2c Bilateral or contralateral lymph node(s) <6cm and ENE(−) N3a Single lymph node >6cm and ENE(−) N3b Single ipsilateral node >3cm and ENE(+) OR multiple lymph nodes of any size, any with

ENE(+) OR single contralateral node of any size and ENE(+)

K. Wong et al.

Distant metastasis (M) M category M criteria

M0 No distant metastasis M1 Distant metastasis

Final staging T N M Stage

Tis N0 M0 0 T1 N0 M0 I T2 N0 M0 II T3 N0 M0 III T1 N1 M0 III T2 N1 M0 III T3 N1 M0 III T1 N2 M0 IV

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Final staging T N M Stage

T2 N2 M0 IV T3 N2 M0 IV Any T N3 M0 IV T4 Any N M0 IV Any T Any N M1 IV

Treatment Recommendations

• Overview

– Prognosis excellent, survival approaches 100% if no spread to other sites – Metastasis rare; common sites=lymph nodes, lungs, bones – 5-year risk of new primary=50% – 5-year recurrence=5% (depending on treatment modality)

• Surgical excision

– Gold standard – 4- to 5-mm margins for low-risk disease (<20mm) – 5- to 10-mm margins for high-risk disease (>20mm)

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Morphoeic: 82% cleared with 5 mm margins, >95% cleared with 13–15mm margins

• Mohs micrographic surgery (MMS)

– Indicated for cosmetically sensitive and high-risk disease (size >2cm, ill-

dened borders, recurrence, PNI, or high-risk anatomic sites such as central face, eyes, nose, lips, ears)

• Radiotherapy (RT)

– Higher recurrence rate than surgery, less acceptable cosmetic outcomes – Primary radiation for nonresectable disease – Adjuvant radiation indicated for positive margins and PNI

• Photodynamic therapy

– Methyl aminolevulinate→cancer cell uptake→protoporphyrin IX→visible

red light→generate free radicals and oxygen species→cytotoxic

– Better cosmesis but higher recurrence rate compared to surgery

• Curettage and cautery

– Indicated for low-risk disease – High recurrence rate; not as commonly used – Consider in small nodular or supercial lesions – Poor outcomes for high-risk lesions

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