Borderline and low malignant potential tumours of soft tissues

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L.D.R. Thompson J.C. Fanburg-Smith B.M. Wenig

Desmoid-type fibromatosis

Definition

A locally aggressive, cytologlcally bland tumour of (myo)fibroblastic phenotype.

ICD-O code 8821/1

Synonyms

Extra-abdominal desmoid, extra-abdominal fibromatosis, desmoid tumour, aggressive fibromatosis, juvenile desmoid-type fibromatosis, infantile fibromatosis.

Epidemiology

Although 15% of cases of desmoid-type fibromatosis occur in the head and neck, the sinonasal tract is uncommonly involved {6,903,2643}. All ages can be affected, especially children {903}. There is a male predilection {903}.

Localization

The maxillary sinus and turbinates are usually affected, and the involvement can occasionally be bilateral {514,826, 903}.

Clinical features

Symptoms include nasal obstruction, epistaxis, mass, facial pain, tooth displacement, and a non-healing tooth extraction site {514,826,903}.

Cular Bland Photo
genized stroma with spindle cells with bland nuclei and elongated vessels.

Macroscopy

The lesion is tan-white, glistening, and rubbery to firm, and is often infiltrative. It measures up to 7 cm. Fibromatosis may be multicentric, especially in the setting of Gardner syndrome {562}.

Histopathology

This is an infiltrative growth with low to moderate cellularity, comprising broad fascicles of bland-looking spindle cells and collagen fibers often arranged in a uniform direction. Elongated blood vessels are frequently observed running parallel to each other.

The spindle cells have a myofibroblastic appearance, with low nuclear to cyto-plasmic ratio and uniformly bland ovoid nuclei with indistinct nucleoli. Mitotic figures are infrequent and never atypical. The matrix is collagenized to focally myx-oid, and keloid-like collagen may be present. The main differential diagnoses include hypertrophic scar and fibrosar-coma.

Diagnosis does not require immunohisto-chemistry, but vimentin and actins are positive. Desmin may be focally positive.

Genetic susceptibility

Fibromatosis can be part of Gardner syndrome {562}.

Prognosis and predictive factors

Fibromatosis can be locally aggressive and involve contiguous structures, with approximately 20% recurrence rate, but it does not metastasize {514,903,2643}. Recurrence generally occurs within the first few years and is related to inadequacy of surgical margins {514,903, 2643}.

Inflammatory myofibroblastic tumour

ICD-O code 8825/1

Inflammatory myofibroblastic tumour uncommonly occurs in the sinonasal tract {2429}. Please see corresponding section in 'Tumours of the hypopharynx, larynx and trachea'.

Glomangiopericytoma (Sinonasal-type haemangio-pericytoma)

Definition

A sinonasal tumour demonstrating perivascular myoid phenotype.

ICD-O code 9150/1

Glomangiopericytoma
Fig. 1.51 Glomangiopericytoma. Characteristic diffuse growth within the submucosa, with effacement of the normal components of the submucosa and preservation of mucoserous glands. The overlying respiratory epithelium remains intact.

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■ S r l'y, »i* ■ ■--■ . . - * ^ * i ,

! 1 f i ■'' i" ? . j ■ * ■ Lí i " r hi '1

Fig. 1.52 Glomangiopericytoma. A A characteristic histomorphologic feature is the presence of prominent perivascular hyalinization. B Tumour cells are ovoid or polygonal. Note the delicate interspersed vascular spaces. C Streaming short spindly cells. Vascular spaces may not be obvious. D Immunostaining for muscle-specific actin is mainly in vessels and focally in tumour cells.

Fig. 1.52 Glomangiopericytoma. A A characteristic histomorphologic feature is the presence of prominent perivascular hyalinization. B Tumour cells are ovoid or polygonal. Note the delicate interspersed vascular spaces. C Streaming short spindly cells. Vascular spaces may not be obvious. D Immunostaining for muscle-specific actin is mainly in vessels and focally in tumour cells.

Synonyms

Sinonasal haemangiopericytoma; hae-mangiopericytoma-like tumour, sinonasal glomus tumour; haemangiopericytoma.

Epidemiology

Sinonasal glomangiopericytomas predilect to the nasal cavity and paranasal sinuses, where they comprise <0.5% of all neoplasms {343,482,2600}. There is a very slight female predominance. All ages can be affected (in-utero to 86 years), but the peak is in the 7th decade.

Localization

Tumours most frequently arise unilaterally in the nasal cavity alone, although extension into paranasal sinuses can occur. Isolated paranasal sinus involvement is uncommon. Rarely, large tumours may appear to arise bilaterally {216,343,482,638,649,681,1364,1779, 1846,2276,2600,2729}.

Clinical features

The majority of patients present with nasal obstruction, epistaxis, or non-specific findings, such as a mass, polyp, difficulty breathing, sinusitis, headache and nasal congestion, present for an average duration of <1 year. Imaging studies show nasal cavity or paranasal sinus opacification by a polypoid mass lesion, frequently accompanied by sinusitis, bone erosion and sclerosis {482,2600, 2729}.

Macroscopy

The generally polypoid tumours range up to 8 cm, with a mean size of about 3 cm. The tumours are beefy red to greyish pink, soft, edematous, fleshy to friable masses, often demonstrating haemorrhage.

Histopathology

This is a subepithelial well-delineated but unencapsulated cellular tumour, effacing or surrounding the normal structures. It is comprised of closely packed cells, forming short fascicles and sometimes exhibiting a storiform, whorled or palisaded pattern, interspersed with many vascular channels. The latter are in the form of capillary-sized to large patulous spaces that may have a "staghorn" or "antler-like" configuration. A prominent peritheliomatous hyalinization is characteristic. The neoplastic cells are uniform, elongated to oval, and possess vesicular to hyperchromatic, round to oval to spindle-shaped nuclei, and lightly eosino-

philic cytoplasm. Mild nuclear pleomor-phism and occasional mitotic figures may be present, but necrosis is not found. Extravasated erythrocytes, mast cells, and eosinophils are nearly ubiquitously present. Occasionally, tumour giant cells, fibrosis or myxoid degeneration may be seen.

Immunohistochemistry

Immunohistochemically, glomangioperi-cytoma is distinctly different from soft tissue haemangiopericytoma by yielding diffuse reactivity for actins, factor XIIIA and vimentin, and lacking strong diffuse staining for CD34. Bcl-2, FVIII-R Ag, CD99 and CD117 are negative {343, 638,1364,2070,2600}.

Differential diagnosis

The differential diagnoses include hae-mangioma, solitary fibrous tumour, glo-mus tumour, leiomyoma, synovial sarcoma and leiomyosarcoma.

Histogenesis

This tumour has been known as haeman-giopericytoma-like tumour or sinonasal haemangiopericytoma, but it is clinically, morphologically and biologically distinct from soft tissue-type or dura-based hae-mangiopericytoma {446,544,773,936, 1723,1724,2276,2386,2689}. The proposed cell of origin is a modified perivas-cular glomus-like myoid cell.

Prognosis and predictive factors

Sinonasal glomangiopericytoma is indolent, with an overall excellent survival (>90% 5-year survival) achieved with complete surgical excision. Recurrence, which develops in up to 30% of cases, may occur many years after the initial surgery {216,343,638,649,2600}. Aggressive-behaving glomangiopericytomas (malignant glomangiopericytomas) are uncommon {216,343,482, 556,1779,2600}, and usually exhibit the following features: large size (>5 cm), bone invasion, profound nuclear pleomorphism, increased mitotic activity (>4/10 high power fields), necrosis, and proliferation index >10% {216,343,1364, 2600}.

Scarring Squamous Cell

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