1、Dermal and Subcutaneous Tumors,Mastocytosis urticaria pigmentosa,Local and systemic accumulations of mast cells Persistent pigmented itchy skin lesions Urticate on mechanical or chemical irritation c-KIT mutation Birth to middle age, 6 mo,Macules, papules, nodules, plaques, vesicles Lesions persist
2、and gradually become chamois- or slate-colored Dariers sign, pruritis Severe symptoms may result from massive liberation of histamine from mast cells after ingestion of known mast cell degranulators Spontaneous resolution is likely in those pts whose disease began in childhood,Urticaria pigmentosa,S
3、olitary mastocytoma,May be present at birth, may develop during the first weeks of life Brown macule that urticates upon stroking Smooth or peau d orange Dorsum of the hand near the wrist Edema, urtication, vesiculation may be observed,Generalized eruption, childhood type Pseudoxanthomatous mastocyt
4、osis Diffuse cutaneous mastocytosis Generalized eruption, adult type Erythrodermic mastocytosis Telangiectasia macularis erupta perstans Systemic mastocytosis Familial urticaria pigmentosa,Giemsa, azure A, or polychrome toluidine blue Local anesthetic adjacent to the lesion, without epi Dx is bx con
5、firmed Histamine metabolites methylhistamine and methylimidazole acetic acid,Prognosis and treatment,In all forms without systemic involvement the prognosis is good Solitary lesions usually involute within 3 years H1 and H2 blockers PUVA Intralesional and topical steroids Avoid physical stimuli,Abno
6、rmalities of neural tissue,Solitary neurofibroma,Soft, flaccid, pinkish white, 2-20 mm Invaginates on pressure, “buttonholing” Solitary or multiple Distinctive histopathologic findings, fibrils, cellular proliferation, and degenerative changes Sx excision,Granular cell tumor,Well-circumscribed, soli
7、tary firm nodule, with a brownish red or flesh tint Usually solitary, 10-15 % multiple 1/3 of cases have occurred on the tongue May occur anywhere on the body Grows slowly Cells stain positively with vimentin, neuron-specific enolase, S-100, and myelin protein Malignant granular cell tumor is rare,N
8、euroma cutis,Three true neuromas exist in the skin and mucous membranes: traumatic neuroma, multiple mucosal neuromas, and solitary palisaded encapsulated neuromas Traumatic neuromas occur commonly on the fingers, tender and painful Multiple mucosal neuromas occur as part of multiple mucosal neuroma
9、 syndrome solitary palisaded encapsulated neuromas occur commonly on the face, resembles BCC,neurothekeoma,Nerve sheath myxoma Benign tumor of nerve sheath Mitotic figures and nuclear atypia are sometimes observed Intradermal or subcutaneous Histologically are divided into two subtypes: myxoid and m
10、ore common cellular variant,schwannoma,neurilemmoma Usually a solitary nerve sheath tumor Most often seen in women Occur almost exclusively along the main nerve trunks of the extremities Soft or firm nodules, may be painful May be multiple May be assoc. with NF-1 or NF-2,Occur in many other organs e
11、xcision,Infantile neuroblastoma,The most common malignant tumor of childhood Cutaneous nodule are most often seen in the younger patients Blue nodules the when rubbed form a halo of erythema Periorbital ecchymoses and heterechromia Good prognosis for patients with skin involvement, spontaneous remis
12、sion,ganglioneuroma,Rarely described in the skin as an isolated entity Arise most often in von Recklinghausens neurofibromatosis Occur in childhood,Nasal glioma,Cephalic brainlike heterotopias Rare, benign congenital tumors Easily confused with hemangiomas Firm, reddish blue lesion on the nasal brid
13、ge No connection with the subarachnoid space Radiography and neurosurgical consultation Does not involute spontaneously,Cutaneous memingioma,Psammoma Results from the presence of meningocytes outside the calvarium Small, hard, fibrous, calcified nodules occurring along the spine, in the scalp, and o
14、n the forehead Usually seen within the first year No distinctive appearance, dx by histo,Encephalocele and Meningocele,Primary defect in the neural tube Present in infancy along the midline Compressible masses that may transilluminate or enlarge with crying Midline masses require intensive radiologi
15、c and neurosurgical evaluation before biopsy,chordomas,Slow-growing, locally invasive Firm, smooth nodules in the sacralcoccygeal region or at the base of the skull Arise from notochord remnants May metastasize late in their course Wide excision and postoperative radiation therapy,Abnormalities of F
16、at Tissue,lipomas,Subcutaneous tumors composed of fat tissue Most commonly found on the trunk Also neck, forearms and axillae Soft, single or multiple, lobulated and compressible Growth to size and remain stationary again be careful of sacrococcygeal lipomas Lesion may be left untreated or excised,S
17、olitary lesions reaching greater than 10 cm should be investigated for malignancy Multiple lesion may be painful if growing rapidly Madelungs disease, benign symmetric lipomatosis Dercums disease, assoc with weakness and psychiatric disturbances,Familial multiple lipomatosis, AD inheritance Bannayan
18、-Riley-Ruvalcaba syndrome MEN 1 Frohlichs syndrome Gardners syndrome,angiolipoma,A painful subcutaneous nodule just slightly above the level of the skin Has all other typical features of a lipoma Seen in young adults who have multiple painful lumps in the skin Multiple subcutaneous angiolipomas have
19、 no invasive or metastatic potential,Neural fibrolipoma,Overgrowth of fibro-fatty tissue Occurs along a nerve trunk and often leads to compression Slowly enlarging subcutaneous mass with tenderness and decreased sensation or parasthesia Median nerve is most commonly involved MRI, no effective treatm
20、ent,Spindle-cell lipoma,Asymptomatic, slow growing subcutaeneous tumor Predilection for the back and neck and shoulders of older men Consists of lobulated masses of mature adipose tissue,Painful Piezogenic pedal papules,Transitory, soft, sometimes painful papules on the sides of the heels Elicited b
21、y weight-bearing and disappearing when this is stopped Occur in at least 75 % of normal individuals Suitable supportive shoes may alleviate discomfort May occur on the wrist,Nevus lipomatosus superficialis,Soft, yellowish papule or ceribriform plaques, usually of the buttock or thigh, less often the
22、 ear or scalp A wrinkled surface characterizes this tumor Onset prior to age of 20,Nevus lipomatosus superficialis,Folded skin with scarring,Rare, aka Michelin Tire Baby Syndrome There are numerous deep, conspicuous, symmetrical, ringed creases around the extremities The underlying skin may manifest
23、 a smooth muscle hamartoma, a nevus lipomatosis, or elastic tissue abnormalities AD, sporadic or an isolated finding assoc with congenital facial and limb abnormalities,Diffuse lipomatosis,Characterized by an early age of onset, by the age of 2, diffuse infiltration of muscle by and encapsulated mas
24、s of mature lipocytes Progressive enlargement and extension Usually involves a large portion of the trunk or extremity,Hibernoma (lipoma of brown fat),A form of lipoma composed of finely vacuolated fat cells of embryonic type Have a distinctive brownish color and a firm consistency Benign and usuall
25、y occur singly Chiefly in the mediastinum and the interscapular region Onset usually in adult life,Pleomorphic lipoma,Occur for the most part on the backs and necks of elderly men Occasional lipoblast-like cells and atypical mitotic figures may require differentiation from a liposarcoma Behave in a
26、perfectly benign manner,Benign lipoblastomatosis,Frequently confused with a liposarcoma Affects exclusively infants and young children, 90% age 3 Commonly involves the soft tissues of the upper or lower extremity A circumscribed and a diffuse form can be distinguished TOC- complete local excision,li
27、posarcoma,One of the less common mesenchymal neoplasms of the soft tissue Usually arise from intermuscular fascia Do not arise from preexisting lipomas Usual course is an inconspicuous swelling of the soft tissue with gradual enlargement When a fatty tumor becomes greater than 10 cm DX should be con
28、sidered Upper thigh is the most common site,Adult males are mostly affected May be well or poorly differentiated Tx is adequate radical excision For metastatic liposarcomas, radiation therapy may be effective,Abnormalities of smooth muscle,leiomyoma,Smooth muscle tumors Characterized by painful nodu
29、les Singly or multiple Benign Treatment is directed toward the removal of the pain source Simple excision is best,Solitary cutaneous leiomyoma Multiple cutaneous leiomyomas Solitary genital leiomyoma angioleiomyoma,Grouped leiomyomata of the back,Congenital smooth muscle hamartoma,Typically a skin c
30、olored or slightly pigmented patch or plaque with hypertrichosis Often present at birth Usually seen on the trunk, lumbosacral area in 2/3 Michelin tire baby syndrome may result from a diffuse smooth muscle hamartoma,Clinically may mimic a mastocytoma, pseudo-Dariers sign is seen in 80% No treatment
31、 is necessary,leiomyosarcoma,Of soft tissue origin are extremely rare May occur as metastasis from internal source Appears in the dermis as a solitary nodule, good prognosis Subcutaneous lesions have a guarded prognosis, with fatal hematogenous metastases in 1/3 WLE or Mohs,Miscellaneous tumors and
32、tumor-associated conditions,Cutaneous endometriosis,Brownish papules in the umbilicus or lower abdominal scars after gynecologic surgery Tender or painful lesions Bluish black from cyclic bleeding Usually misdiagnosed as malignant metastases Surgical excision Preoperative tx with danazol or leuproli
33、de may reduce size,teratoma,May develop in the skin but are most common in the ovaries or testes No characteristic clinical features Tissue representing all three germ layers are present Occasionally malignancy may occur,Metastatic carcinoma,5 to 10% of patients with cancer develop skin metastases U
34、sually present as numerous firm, hard, or rubbery masses Predilection for chest, abdomen or scalp Sister Mary Joseph nodule, metastatic tumor localized to the umbilicus, most common primary sites include the stomach, large bowel, ovary and pancreas,A poor prognosis is usually the rule The involvemen
35、t of the skin is likely to be near the area of the primary tumor Breast cancer is the type most commonly metastatic to the skin in women and melanoma followed by lung cancer in men Metastatic lesions are uncommon in children,Paraneoplastic syndromes,Some cancers produce findings in the skin that ind
36、icate to the clinician that an underlying internal malignancy may be present Bazexs syndrome, characterized by violaceous erythema and scaling of the fingers, toes, nose, and aural helices. Secondary to a primary malignant neoplasm of the upper aerodigestive tract,Bazexs syndrome,Necrolytic migrator
37、y erythema, seen with glucagon-secreting tumors of the pancreas Erythema gyratum repens, erythema with characteristic wood-grain-pattern scales, is almost always associated with and underlying malignancy Hypertrichosis lanuginosa aquisata, most common with lung and colon ca,EGR,Erythema gyratum repe
38、ns,Hypertrichosis lanuginosa,The sign of Lesser-Trelat, the sudden appearance of multiple pruritic seborrheic keratosis, associated with and internal malignancy Trousseaus sign, migratory thrombophlebitis, pancreatic ca Pityriasis rotunda Tripe palms Several others with less frequency,carcinoid,Char
39、acterized by distinctive involvement of the lungs, heart, gastrointestinal tract and the skin Cutaneous flushing lasting 5-10 minutes Involves the head and neck producing a scarlet color Cyanosis may be present Episodic flushing continues for months or years,The release of excessive amounts of serot
40、onin and bradykinen into circulation produces attacks of flushing of the skin, weakness, abdominal pain, nausea and vomiting, sweating, palpitation, diarrhea and collapse Tumor arises from the argentaffin Kulchitsky chromaffin cells of the appendix or terminal ileum (gi, lungs, ovaries, testes),The diagnosis may be established by finding high levels of 5-hydroxyindolacetic acid (5-HIAA) in the urine Tx- primary tumor should be removed, and excision of metastatic lesion should be considered Chemotherapy,
