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Fig. 1.55 A Myxoma. The stellate cells have thin processes which extend out into the background mucinous matrix. B Vascular leiomyoma. Spindle tumour cells are identified scrolling off thick muscle-walled vessels.

ICD-O code

9120/0

Synonyms

Lobular capillary haemangloma; pyogenic granuloma; capillary haemangloma; cavernous haemangloma; epulis gravidarum.

Epidemiology

Mucosal haemangiomas of the nasal cavity, paranasal sinuses and nasopharynx account for 10% of all head and neck haemangiomas and approximately 25% of all non-epithelial neoplasms of this anatomical region. The haemangiomas occur in all ages, although there is a peak in children and adolescent males, females in the reproductive years, and then an equal distribution beyond 40 years of age. Patients with cavernous haemangiomas tend to be men in the 5th decade {166,167,658,823,1037,1189, 1270,1738,2056,2333}.

Etiology

Lobular capillary haemangioma (pyogenic granuloma) has an association with injury and hormonal factors (pregnancy or oral contraceptive use) {2158}.

Localization

The septum is most frequently affected (specifically, the anterior septum in Little's area), followed by the turbinate (usually the tip) and the sinuses {658, 823,1037,1189,1270,1738,2056,2753}.

Clinical features

Patients present with unilateral epistaxis and/or an obstructive painless mass. Sinus lesions present as sinusitis, prop-tosis, mass, anaesthesia or pain.

Symptoms are usually present for a short duration {658,823,1037,1189,1270,1738, 2056,2333,2753}.

Macroscopy

The tumours range up to 5 cm, with a mean size of <1.5 cm. Grossly, they appear as a red to blue submucosal soft, compressible, flat or polypoid lesion, often with an ulcerated surface. Cavernous haemangiomas are spongy on sectioning {658,823,1037,1189,1270, 1738,2056,2333,2753}.

Histopathology

Haemangiomas are usually localized and can be divided into capillary and cavernous types based on the size of the blood vessels. Haemangiomatosis is a more diffuse lesion often involving contiguous structures {823,1270,2126}. Lobular capillary haemangioma is a circumscribed lesion comprising lobules of capillaries lined by plump endothelial cells and supported by prominent peri-cytes. The lobules are separated by a fibromyxoid stroma. The cellularity of the lobules may be quite high. Mitotic figures are often observed, but are never atypical. The surface epithelium often forms collarettes around the lesion {658, 823,1037,1189,1270,1738,2056,2333}. If the lesion is ulcerated and inflamed, the term 'pyogenic granuloma' has been applied.

Cavernous haemangiomas are frequently intraosseous or involve the turbinates or lateral nasal wall. They are composed of multiple, large thin-walled, dilated blood vessels separated by scant fibrous stroma {658,823,1037,1189,1270,1738, 2056,2333}.

Venous haemangiomas are composed of thick-walled veins with abundant smooth muscle, but rarely occur in this location.

Immunoprofile

The tumour cells are immunoreactive for Factor VIII related antigen, CD34, CD31 and Ulex europaeus I lectin. The proliferated blood vessels are enwrapped by actin-positive pericytes.

Differential diagnosis

Haemangiomas should be distinguished from granulation tissue, telangiectasia, vascular malformations, vascular polyps (haemorrhagic type), papillary endothe-lial hyperplasia, angiofibroma, bacillary angiomatosis, angiolymphoid hyperpla-sia with eosinophilia, glomus tumour, sinonasal glomangiopericytoma, lymphangioma, Kaposi sarcoma, and angiosarcoma. Haemangioma can be distinguished from granulation tissue by the lobular arrangement of the capillaries in the former and the more parallel arrangement of vessels in the latter. The distinction between a haemangioma and telangiectasia may be difficult but is facilitated in a patient with a known family history of hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) {658,823,1037,1 189,1270,1738,2056, 2073,2333,2600}.

Prognosis and predictive factors

Haemangiomas are generally easy to remove, although larger tumours may be complicated by excessive bleeding. They should be removed in all ages, especially in children since aplasia of the nasal cartilages may cause eventual disfigurement. If the tumour is pregnancy-

Fig. 1.56 Haemangioma. A A lobular arrangement around large patulous vessels is seen in this lobular capillary haemangioma. B Cavernous haemangioma with large, dilated vascular spaces and an intact surface epithelium.

Fig. 1.56 Haemangioma. A A lobular arrangement around large patulous vessels is seen in this lobular capillary haemangioma. B Cavernous haemangioma with large, dilated vascular spaces and an intact surface epithelium.

Fig. 1.57 Schwannoma. A A well circumscribed spindle cell neoplasm has focal degeneration with a lymphoid cuff. B Nuclear palisading around fibrillar cellular processes is characteristic for a peripheral nerve sheath tumour. C Hyalinized vessel next to a Verocay body with palisaded nuclei.

: *■ " ''rniF" J k "i^' VV i — « ■ .''W^v-'ii^ ^r-v

: -v-^IÇig^ ? 7. -, .. • /V-L V - . .■£> V . ^ . ^ V' -"' A ■ -u

Fig. 1.57 Schwannoma. A A well circumscribed spindle cell neoplasm has focal degeneration with a lymphoid cuff. B Nuclear palisading around fibrillar cellular processes is characteristic for a peripheral nerve sheath tumour. C Hyalinized vessel next to a Verocay body with palisaded nuclei.

related, regression will often occur after parturition. Multiple recurrences are more common in children if the lesional bed is not completely eradicated.

Schwannoma

Definition

A usually encapsulated, benign tumour composed of differentiated, neoplastic Schwann cells.

ICD-O code

9560/0

Synonyms

Neurilemoma, neurilemmoma, peripheral nerve sheath tumour.

benign

Epidemiology

Less than 4% of schwannomas involve the nasal cavity and paranasal sinuses {1091,2351}, and they occur in middle-aged adults with an equal gender distribution {2351}.

Localization

Sinonasal schwannomas arise from the branches of the trigeminal (5th) nerve and autonomic nervous system, and most commonly involve the ethmoid and maxillary sinuses, followed by the nasal cavity, sphenoid and frontal sinuses {1023,1091,2018,2351}. Cellular schwannoma tends to be located in the midline.

Clinical features

The presenting symptoms include obstruction, rhinorrhea, epistaxis, anosmia, headache, dysphagia, hearing loss, facial or orbital swelling, and pain {2018,2351}.

Macroscopy

Sinonasal schwannoma ranges in size up to 7 cm. It is a well-delineated but non-encapsulated globular, firm to rubbery yellow-tan mass. The cut surfaces show tan-grey, yellowish, solid to myxoid and cystic tissue, commonly with haemorrhage.

Tumour spread and staging

The tumour can expand into the orbit, nasopharynx, pterygomaxillary fossa and cranial cavity {2351}.

Histopathology

Schwannoma is composed of cellular Antoni A areas with Verocay bodies and hypocellular myxoid Antoni B areas. The cells are fusiform with elongated fribillary cytoplasm, and buckled to spindled nuclei which show little pleomorphism, although scattered large pleomorphic or bizarre cells can be present in some cases. Nuclear palisading is often evident in some foci. There are frequently small to medium-sized vessels with ecta-sia, thrombosis and perivascular hyalin-ization in the Antoni B areas. Extensive degenerative changes can occur, and may result in only a thin rim of recognizable tumour. Cellular variants exhibit only the Antoni A pattern, but no fascicular growth or Verocay bodies.

Immunoprofile

The tumour cells are strongly and diffusely immunoreactive for S100 protein. CD34 only stains some more slender cells in the Antoni B areas. Neurofilament is absent. GFAP and keratins may be positive.

Prognosis and predictive factors

Schwannoma is a benign tumour with a very low recurrence potential. Malignant transformation is exceptional {1690}.

Neurofibroma

Definition

A benign tumour of peripheral nerve sheath phenotype with mixed cellular components, including Schwann cells, perineurial hybrid cells and intraneural fibroblasts.

ICD-O code

9540/0

Epidemiology

Neurofibromas are extremely rare in the sinonasal tract. In NF1-related neurofibromas, patients tend to be younger, with a male predominance {2745}. For the more common sporadic neurofibromas,

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