CHAPTER 22

HEMANGIOMA AND SOME MESENCHYMAL TUMOR

(血管腫と間葉系腫瘍)

 

I. Hemangioma(血管腫)

A.   Classification of Benign and Malignant Hemangioma

1.    Neoplastic growth

 a.  Spontaneously disappearing group

1)   Strawberry mark(いちご状血管腫)

2)   Multiple congenital hemangiomatosis

 b.  Non spontaneously disappearing group

1)   granuloma teleangiectaticum(血管拡張性肉芽腫)
= pyogenic franuloma(化膿性肉芽腫)

2)   angioblastoma Nakagawa(血管芽細胞腫)

3)   Kasabach-Meritt syndrome(カサバッハ・メリット症候群)

4)   Glomus tumor(グロムス腫瘍)

2.    Mainly dilating blood vessel growth

 a.  Spontaneously disappearing group

1)   salmon patch(サモンパッチ)

2)   cutis marmorata teleangietatica congenita
(先天性血管拡張性大理石様皮斑)

 b.  Non spontaneously disappearing group

1)   portwine stain(赤酒しみ様血管腫)

2)   cavernous angioma(海綿状血管腫)

3)   cirsoid aneurysm

4)   angiokeratoma(被角血管腫)

・ mibelle

・ scroti

・ corporis circumscriptum naeriforme

・ solitary

5)   angioma serpiginosum(蛇行状血管腫)

6)   angioma senilis(老人性血管腫)

B.   Classification of malignant hemangioma

1.    Hemangiopericytoma: borderline and malignant

2.    Malignant Hemangioendothelioma

3.    Kaposi's Sarcoma...etc.

II. Clinical and Histopatholigical Characteristics of Hemangiomas

A.   Neoplastic growth, spontaneously disappearing group

Strawberry Mark (Capillary Hemangioma of Infancy)(いちご状血管腫)

 a.  A soft, bright-red to deep-purple, vascular nodule-to-plaque that develop at birth or soon after birth and disappears spontaneously by the fifth year.

 b.  Large straw-berry mark, usually associated with cavernous hemangioma may have platelet entrapment and thrombocytepenia (Kasabach-Merritt syndrome) developing thrombocytepenic purpura: anemia, splenomegary, bleeding from internal organ.

B.   Neoplastic growth, non spontaneously disappearing group

Glomus Tumor(グロムス腫瘍)

1.    Types:

a)   solitary type; a purple nodule (a few mm) is tender, with severe proximal radial pain. Most commonly situated in the nail bed.

b)   Multiple type (glomangioma): Some instances dominantly tran mitted. The lesions, located intracutaneously or subcutaneously, appear either at one area or more commonly wide spread pattern. As a rule, asyptomatic.

2.    Histogenesis

Both solitary and multiple types are growth of arterial segment of the cutaneous glomus, a smooth muscle cell.

C.   Mainly dilating blood vessel growth, spontaneously disappearing group

Cutis Marmorata Teleangietatica Congenita (congenital phlebectasia)(先天性血管拡張性大理石様皮斑)

1.    Skin Lesions:

Characterized by exaggerated cutis marmorata with generalized or segmental distribution, phlebectasia, teleangiectasia, and ulceration, usually on the extremities.

2.    Complications:

Varicosities, hemangiomas, and hypoplasia and hypertrophy of soft tissue and bone.

D.   Mainly dilating blood vessel growth, non spontaneously disappearing group

Port-Wine Stain (PWS)(赤酒しみ様血管腫)

1.    A congenital disease, an irregularly shaped, red or violaceous, macular vascular malformation of dermal blood vessels which is present at birth and never disappear spontaneously.

2.    Clinical Variant

 a.  Nevus flammeus (salmon patch) locates on the nuchal area or glabella. It tends to regress spontaneously.

 b.  Sturge-Weber syndrome (SWS) is the association of PWS at trigeminal nerve area with vascular malformation in the eye (glaucoma) and leptomeniges, and superficial calcification of the brain.

 c.  Klipple-Trenaunay-Weber syndrome may have an associated PWS overlying the deeper vascular malformation of soft tissue and born, resulting in their hypertrophy.

 d.  Systemic Symptoms in SWS

1)   contralateral hemiparesis

2)   muscular hemiatroply

3)   epilepsy

4)   mental retardation, glaucoma and occular palsy.

Cavernous Hemangioma (CH)(海綿状血管腫)

1.    Skin Lesions:

A deep vascular malformation, characterized by soft compressible deep-tissue usually red- purple or blue swelling or tumor.

2.    Variants of CH

 a.  Blue Rubber Bleb Nevus

1)   Spontaneously painful or tender, soft blue nodule or swelling (few mm-several cm) in dermis and subcutaneous tissue.

2)   Oral mucosa and GI tract may be involved and the lesion may be a source of hemorrhage.

 b.  Maffucc's Syndrome

CH associated with dyschondroplasia,manifested as hard nodules on finger or toe, bony deformity.

E.   Malignant hemangioma(悪性血管内皮細胞腫)

Malignant Hemangioendothelioma

1.    Etiology:

 a.  A striking predilection for the skin of the head.

 b.  Most patients are in the seventh to eighth decade

2.    Skin Lesions

The bluish or purple color, somewhat reminiscent of a bruise or contusion, the frequent occurrence of a peripheral erythema and of satellite tumors, small foci of intratumorous hemorrhage with spontaneous bleeding.

3.    Clinical Course

The most striking feature:

 a.  Tendency for extensive local spread

 b.  Distant metastasis to lung and cervical lymph nodes

4.    Histology

 a.  Unrestrained proliferation of atypical endothelal cells

 b.  Very free anastomosis of the tumor vessels.

 c.  Tumor cells appear large, pleomorphic, cuboidal or spindle-shaped, and invade between collagen bundle, with cords or occasional slit-like vascular lumina.

 d.  Hemorrhage is frequent.

5.    Treatment

 a.  administration of IL-2

 b.  radiation

Kaposi's Sarcoma (KS)(カポジ肉腫)

1.    Epidemiology; Epidemic or AIDS-associated.

2.    Clinical Lesions:

A multi-centric systemic vascular tumor characterized by violaceous nodules and by edema secondary to lymphatic obstruction.

3.    Clinical Variants of KS

 a.  Classic KS

 b.  KS with iatrogenic immunosuppresion: transplantation recipients

4.    Etiology:

Unknown. Evidence points to an infective agent, bpossibly a retrovirus, other than HIV.

5.    Cutaneous lesions

 a.  Most often begin as an ecchymotic-like macule, and evolve violaceous, red or tan macules, papules, plaques, tumors located in the dermis or hypodermis.

 b.  Erosion or ulcer may develop.

 c.  Progressive edema of involved extremities can be associated with progressive fibrosis and stricture limiting movement.

6.    Distribution of lesions

Widespread trunk, head, nose, ears, penis, anus, legs, palms and soles.

7.    General Examination

 a.  Lung: pulmonary infiltrates resulting in progressive repiratory  failure

 b.  GI tract: GI hemorrhage, rectal obstruction

8.    Diagnosis

Confirmed on skin biopsy

9.    Histology

 a.  Early, proliferation of blood vessels lined by more or less atypical endethelial cells with admixture of histiocytes, lymphocytes and plasma cells.

 b.  Olderly, discret intradermal nest-like mass with vascular channels of atypical endothelium and spindle-shaped cells, with decrement of inflammatory cells.

 

III. Mesenchymal Tumors

A.   Classification of Mesenchymal Tumor

●線維組織球性腫瘍

 dermatofibroma(皮膚線維腫)

 juvenile xanthogranuloma(若年性黄色肉芽腫)

 dermatofibrosarcoma protuberans(隆起性皮膚線維肉腫)

 malignant fibrous histiocytoma(悪性線維性組織球腫)

 giant cell tumor of tendon sheath(巨細胞性腱腫瘍)

●良性間葉系腫瘍

 soft fibroma(軟性線維腫)

 neurofibroma(神経線維腫)

 keloid(ケロイド)

 leiomyoma cutis(皮膚平滑筋腫)

 lipoma(脂肪腫)

 mastocytoma(肥満細胞腫)

 lymphangioma(リンパ管腫)

●悪性間葉系腫瘍

 fibrosarcoma(線維肉腫)

 neurofibrosarcoma(神経線維肉腫)

 leiomyosarcoma(平滑筋肉腫)

 liposarcoma(脂肪肉腫)

 rhabdomyosarcoma(横紋筋肉腫)

 リンパ腫/白血病

 

B.   Malignant Fibrous Tissue Tumors

Malignant fibrous histiocytoma (MFH)(悪性線維性組織球腫)

・ A pleomorphic tumor that consists of two types of undifferentiated cells; histiocytic and  fibroblastic cells.

・ This tumor most common among the other soft tissue tumors, and develops in 40〜 60 decades usually of the extremities.

1.    Histopathology:

Consisting of atypical histiocytic cells, bizarre giant cells and fibroblastic cells, with storiform pattern (storiform-pleomorphic type).  mitosis : frequent.

2.    Treatment:

Wide and deep excision

C.   Dermatofibrosarcoma protuberances(隆起性皮膚線維肉腫)

1.    Age: Typically arise in the third and fourth decades, and is uncommon in the very young old.

2.    Clinical Lesions:

 a.  Presents as a multi nodular mass which is slow-growing with predilection for the trunk and thighs, and shows reddish-blue discoloration.

 b.  Ulceration may occur.

 c.  Local recurrence after excision occurs in about thirty percent of cases.

 d.  Metastasis is rare.

5.    Histopathology

a)   Fibroblastic elongated spindle cells are densely located entire dermis and into the subcutis.

b)   The cells are characteristically arranged in a storiform pattern.

c)    Mitosis and multi nucleated giant cell : infrequent.

6.    Variant

Pigmented dermatofibrosarcoma (Bednar's tumor):

Contains small numbers of melanin forming dermal dendritic cells.

7.    Treatment:

Wide and deep excision