Chapter 34: SOFT TISSUE

TUMORS
Soft Tissue Tumors
 SOFT TISSUE TUMORS
Soft tissue tumors are those originating in the supportive and connective
tissues of the body, other than bone or cartilage. Benign and malignant forms
occur. In addition, there are tumor-like lesions (“pseudotumors”) that may
clinically suggest a neoplasm. The difference is based upon a good history,
examination and biopsy
Soft Tissue Tumors
1. Scar (cicatrix), Hypertrophic scar, Keloid:
a. Reactive connective tissue lesions, secondary to penetrating trauma, and
therefore secondary lesions (see Chapter 13, Dermatology)

2. Fibroma:
a. A benign, self-limiting proliferation of fibroblasts, producing collagen, most
being subcutaneous single lesions
b. They occur at any age
c. Koenen's Periungual Fibromas are seen on the hands and feet of about
one-third of patients with tuberous sclerosis

NOTE* Patients with tuberous sclerosis show an acneform facial rash whose
lesions are called adenoma sebaceum, but which are angiofibromas. Some
patients also are epileptic due to intracranial calcifications. This form of
tuberous sclerosis is called Epiloia. Fibromas may develop independent of
other diseases and are firm, discrete lesions. Sometimes other
elements are involved, e.g. fibrolipoma, fibrokeratoma, angiofibroma,
fibromyxoma, xanthofibroma, neurofibroma, etc. Lesions may recur after
excision

3. Fibromatosis: This is a group of diseases demonstrating infiltrative

growth that makes them less discreet than fibromas
a. Lesions are frequently multiple, and patients may have more than one
type
b. Plantar fibromatosis usually affects the medial fascial slip, but any area
may be affected. This is correctly called Ledderhose's or Dupuytren's disease
c. A cure may be impossible because the lesion is infiltrative so that residual
unseen lesion is always left behind after excision
d. Variants affecting the feet may include juvenile hyalin fibromatosis, extra-
abdominal desmoid tumors, infantile digital fibromatosis, and juvenile
aponeurotic fibromatosis of Keasbey (which also occurs in adults)
e. Desmoids also occur in Gardner's syndrome
f. Fibromas may histologically resemble well diferentiated fibrosarcoma
g. Older Keasbey lesions may become chondrified or ossified. Fibroosseous
pseudotumor of the digits affects the fingers and toes, showing soft tissue
ossifications. It is not the same as a Keasbey lesion
 NOTE* A differential diagnosis of fibrosarcoma vs. plantar fibromatosis should
be kept in mind on initial examination of the foot

4. Myxomas:
a. Are soft tumors of fibrous origin that show a loose fibrous stroma and
much mucin and mucopolysaccharide within
b. This group includes digital mucus cysts and the synovial cysts such as
ganglion (only when the tendon sheath is present, hence, never distal to the
tarsus), and bursa
c. Synovial cysts can also be intraosseous lesions
d. This group are pseudotumors

5. Fibrosarcoma:
a. A malignant, collagenous, metastatic tumor that can occur at any age and
which is uncommon in the foot
b. They may be deep or intradermal
c. They also may be well differentiated or poorly differentiated
d. They may show a histologic "herringbone" pattern of cellular arrangement

6. Histiocytomas:
a. Are similar to fibromas, but show less collagen
b. The histiocyte is considered a facultative fibroblast, a cell state (rather
than a true cell type)
c. There are 3 major groups:
i. Fibroblastic fibrous: includes the following types
• Dermatofibroma
• Xanthomas (some)
• Myxomas
• Dermatofibrosarcoma protuberans (malignant): show a storiform
pattern
histologically
ii. Histiocytic fibrous:
• Giant cell tumor of soft tissue (and bone)
• Xanthogranuloma
• Pigmented villonodular synovitis
• There are also malignant forms recognized
iii. Pleomorphhic fibrous: A group of unusual xanthomas,
xanthogranulomas, and atypical fibroxanthomas

NOTE* Tendon xanthomas may suggest lipoproteinemia. Necrobiotic

xanthogranulomas may be associated with multiple myeloma. Multicentric
reticulohistiocytosis may be a cause of polyarthritis

7. Synovial Chondromatosis (loose bodies, joint mice, joint rice,

osteochondritis dissecans):
a. A rare condition that follows trauma to synovium of large joints such as the
ankle
b. Some of the bodies may ossify
c. X-rays and scans are helpful in the diagnosis

8. Synovial sarcoma (tendosynovial sarcoma):

a. A highly malignant tumor of synovial tissues, often not arising from a joint
b. Young people are affected
c. They may be slow or fast in growing and are very metastatic, especially if
5 cm or larger in size
d. Radiographic techniques and biopsy aid in the diagnosis
e. Treat aggressively (chemotherapy, surgery, irradiation)
f. This is one of the few tumors for which there is no benign equivalent (i.e.
there is no synovioma)

9. Subcutaneous Nodular Fat Necrosis (pancreatic panniculitis):

a. Consists of reddish, tender nodules on the feet and ankles often
accompanied by ankle arthralgias
b. This accompanies pancreatitis and pancreatic carcinoma
c. Elevated blood levels of trypsin increase vascular permeability so that
elevated serum lipase escapes and damages the fat, causing inflammatory
tumor-like nodules
d. If biopsy proven, these lesions suggest need for immediate consultation

10. Lipogranulatomatosis (Farber's disease):

a. Very rare, recessively inherited sphingolipidosis, showing periarticular and
tendon masses, especially at the wrists and ankles
b. Other organs are affected and patients rarely live beyond the first year
c. The masses may, therefore, be diagnostic, requiring immediate
consultation

11. Piezogenic Papules:

a. Represent cystic herniations of subcutaneous heel fat into the dermis
b. They are seen on weightbearing in obese patients
c. They may be painful and can undergo necrosis
d. Control with heel cupping devices and diet

12. Lipoma:
a. A benign tumor of adipose tissue and multiple lesions may occur
b. These may be present independently or as part of Gardner's syndrome and
neurofibromatosis-1
c. Lipomas do not reduce in size as fat is lost due to diet or illness
d. Variants include fibrolipoma, angiolipoma, and myelolipoma (producing
myeloid hematopoietic cells)
e. They can be excised locally without recurrence
f. Tendon sheath lipomas (endovaginal lipomas) may occur in the feet and/or
hands
g. Lipoblastoma may affect the feet of children
13. Liposarcoma:
a. Is a malignant, metastatic tumor of fatty tissue
b. It is probable the most common lower extremity sarcoma in adults, but it is
uncommon in the feet
c. There are gradations from well to poorly differentiated
d. They may be slow growing and asymptomatic until a nerve or other tissue
is sufficiently affected to result In pain or altered function e. These are deep
seated tumors that should be excised followed by chemotherapy and
irradiation

14. Myositis Ossificans (Munchmeyer's disease):

a. A benign reactive lesion deep in striated muscle resulting from trauma
b. Early stages may be hard to tell on x-ray and histologically from the
parosteal or extraosseous forms of osteogenic sarcoma
c. Older lesions appear fully ossified and may be excised if they impede
function or cause pain
d. Myositis ossificans progressiva is a rare hereditary disease of children and
young adults in which ossified lesions affect many tissues and are not related
to trauma (patients may show shortened digits, absent thumbs and great
toes, or hallux valgus bilaterally)

15. Rhabdomyoma:
a. Is a benign tumor of striated muscle and has not been reported in the foot
b. Patients showing signs and symptoms of tuberous sclerosis (see Fibroma
section this chapter) may have cardiac rhabdomyoma and should, be sent for
examination

16. Supernumerary Muscles:

a. Have been reported in the ankle region and may simulate tumors

17. Rhabdomyosarcoma:
a. Very malignant metastatic tumor of striated muscle
b. Rare in the foot, there have been reported cases
c. There are 2 main types:
i. Embryonal: seen in mostly younger persons
ii. Pleomorphic: seen in older persons
d. These tumors are fast-growing and they erode bone
e. Amputation, chemotherapy, and irradiation are all treatment methods

NOTE* Probably the most common lower extremity malignant tumor of

children
18. Leiomyoma:
a. Is a benign tumor arising from smooth muscle
 NOTE* The sources of smooth muscle in the feet are the arrector pill muscles
associated with pilosebaceous units in the dorsal skin of the feet and toes,
and blood vessels (frequently veins) anywhere in the foot.

b. The tumors are pilar leiomyomas and angioleiomyomas

c. These lesions are often painful
19. Leiomyosarcoma:
a. A malignant smooth muscle tumor rare (but does occur) in the ankle or
foot area
b. Lesions may be superficial (dermal in origin) or deep (subcutaneous)
c. Metastases from other sites may occur in the foot

20. Neuromas:
a. Are not tumors
b. They are reactive lesions arising as a result of trauma
c. Rudimentary (supernumerary) digits may contain neuromatous tissues

21. Schwannoma:
a. Is the benign peripheral nerve sheath tumor seen in the lower extremities
b. It is considered a more accurate term than the older "neurilemoma" or
"neurinoma"
c. Slow growing, sometimes painful and mainly occurring on the flexor
surfaces
d. They may fluctuate due to cystic changes
e. These fall into the peripheral nerve sheath tumor (PNST) group
f. Histologically one sees Verocay bodies that may be well developed (Antoni
type A) or that may show myxoid degeneration (Antoni type B)
g. The "ancient schwannoma" is one that shows cystic degeneration and/or
calcification
h. These lesions can be excised and are not recurrent
i. In association with neurofibromatosis-1 there may be multiple
schwannomas
j. The neurothekeoma is a benign peripheral nerve sheath tumor

22. Neurofibroma:
a. A benign, slow-growing, solitary, superficial, usually asymptomatic nodule
b. Excision is curative
c. Most cases occur in women
d. Neurofibromatosis-1 (von Recklinghausen's disease, multiple
neurofibromatosis) is a hereditary malformation involving neural, cutaneous,
and fibrous elements
i. Many organ systems may be involved
ii. There are strict criteria for diagnosis of neurofibromatosis-1 that involve
cafe-au-lait spots
iii. Patients show a high rate of development of unrelated malignant tumors
iv. There is a high risk of transformation of neurofibromas to
neurofibrosarcomas in neurofibromatosis-1
v. Diseases associated with neurofibromatosis-1 include:
• Scoliosis
• Erosions of bone (due to neurofibromas)
• Frohlich's syndrome
• Addison's disease
• Endocrine tumors
• Increased incidence of malignant melanoma
• Connective tissue tumors
• Multiple lipomas
• Meningocele and syringomyelia

23. Malignant Peripheral Nerve Sheath Tumors (MPNST):

a. These include malignant schwannoma and malignant neurofibrosarcoma
b. These are extremely rare in the feet but are a risk in the patient with
neurofibromatosis-1 (when present there is a high mortality rate
c. These tumors may also arise as a result of overexposure to irradiation
(therapeutic or occupational)
d. These tumors are metastatic and may be painful
e. Treatment consists of amputation (improvements in chemotherapy and
irradiation are still being assessed)
f. Malignant triton tumor is a malignant schwannoma with evidence of
concurrent rhabdomyosarcoma

24. Malignant Neuroepithelioma (adult peripheral neuroblastoma,

primative neuroectodermal tumor):
a. Rare in the feet but has been reported
b. Neuroectodermal tumor of infancy (pigmented neuroectodermal tumor of
infancy, melanotic progonoma) is also extremely rare in the foot but has
been reported

25. Granular Cell Tumor (Abrikossoff tumor):

a. Occurs in benign and malignant forms and is uncommon
b. It was once thought to be a tumor of muscular origin, but has been shown
to be of peripheral nerve origin
c. It does occur in the foot

26. Angiomas:.
a. May arise from the endothelium of blood vessels and/or lymphatic vessels
b. Predominant picture based on the presence of blood cells determines
whether the lesion is a hemangioma or a lymphangioma

27. Congenital Hemangiomas:

a. May be localized or may involve large areas of limb and or other organs
b. Most are capillary hemangiomas and after what appears to be a spurt of
rapid growth (in the first 6 months of life) most involute within a year or two
c. Patients should be checked to determine whether a larger (venous)
hemangioma underlies the surface lesion
d. There are rare cases in which capillary hemangiomas in infants are
accompanied by thrombocytopenia (Kasabach-Merritt syndrome)
28. Acquired Tufted Hemangioma:
a. Is the adult form of congenital capillary hemangioma
b. Verrucous hemangioma (angiokeratoma circumscriptum) underlies
hyperkeratoses and may clinically resemble warts or angiokeratoma
c. Cherry angiomas (De Morgan spots, senile angiomas) are tiny papular
angiomas that arise (mostly on the trunk) after adolescence
d. Nevus flammeus (nevus telengiectaticus, “port wine stain”) is a dermal
lesion with many dilated capillaries that usually suggests an underlying
vascular anomaly such as arteriovenous malformation and/or varicosities in
the Parkes-Weber syndrome (osteohypertrophic hemangiectasia, Klippel-
Trenauney syndrome). The A-V malformations usually produce bruits audible
with a stethoscope, local temperature changes, limb hypertrophy, and a risk
of “high output” heart failure

29. Cavernous Hemangiomas:

a. Composed of arterial and venous channels and are deep, large, diffuse
lesions that may clinically feel like a “bag of worms”
b. X-rays may show intravascular calcifications called phleboliths (associated
with thrombi)
c. Venous hemangiomas are clinically similar but do not show the arterial
elements
i. These types of hemangiomas are seen in the blue rubber bleb nevus,
Maffuci (Kast-Maffuci) syndrome, ataxia telangiectasia, von Hippel-Lindau
disease, and Sturge-Weber syndrome

30. Angiokeratoma:
a. The name given to a number of syndromes that affect the feet (and
other areas) showing dilated capillaries (ectasias) with secondary epidermal
hyperplastic changes
b. There are various types, all (except the Fordyce type) affecting the feet
i. Mibelli type
ii. Papular type
iii. Fabry type

NOTE* Angiokeratoma of Fabry is a storage disease whereas the others are

not

c. Cobb's syndrome involves a cutaneous hemangioma in a dermatomal

distribution resembling angiokeratoma and associated with a spinal cord
angioma

31. Angioma Serpiginosum:

a. Consists of red, flat, grouped, asymptomatic, dilated capillaries, forming
net-like or macular patterns
b. Most occur in postadolescent women

32. Pyogenic Granulomas:

a. Eruptive hemangiomas that are friable, easily traumatized, and that may
become secondarily infected
b. Most are on a stalk

33. Glomus Tumors (glomangiomas, angiomyoneuromas):

a. Arise in the digits as a tiny, painful and tender, frequently subungual
tumors
b. They arise from the Suquet-Hoyer canal, the shunts arising from arterioles
that bypass the capillary network to the venules
c. They may be red to purple and may be accompanied by thrombocytopenia
(hence a variant of the Kasabach-Merritt syndrome)
d. Most occur as single lesions
e. Glomangiosarcoma is extremely rare, but has been reported

34. Synovial Hemangiomas:

a. Arise in joints and tendon sheaths
b. Rare and difficult to excise
c. There is pain, swelling, joint effusion and hemarthrosis

35. Hemangiopericytoma:
a. Rarely occurs in the feet
b. There are benign and malignant forms, and histologic characteristics do
not necessarily correlate with the benign or malignant nature of the lesion
c. The patients must be watched for recurrence and/or metastases

36. Lymphangiomas:
a. As a group are rare
b. They may arise in the context of chronic and extensive lymphedema, e.g.
Milroy's disease, elephantiasis due to Wucheria infestation, etc.
c. Cystic hygroma represents a cavernous lymphangioma

37. Papillary Endothelial Hyperplasia (Masson's

pseudoangiosarcoma):
a. An intravascular proliferation of endothelial cells in association with a
thrombus, and usually follows trauma
b. It is not common
c. Most cases show single lesions and these may be difficult to differentiate
histologically from angiosarcoma

38. Ectasias (vascular dilatations):

a. May occur in the feet and be mistaken for angiomas
b. The most common is the “spider nevus” (spider angioma, nevus araneus)

39. Angioendothelioma:
a. The name given to group of lesions that are considered by some to be of
intermediate malignancy
b. Types:
i. Spindle cell type:
• Is most apt to occur on the lower extremities
• Uncommon, seen frequently in young males
ii. Reactive proliferating angioendotheliomatosis:
• A vascular, benign lesion in response to infection, (especially subacute
bacterial endocarditis) that truly involves endothelial proliferation
• Usually clears n 6-24 months
• AIDS patients may develop this lesions n response to cat scratch fever
organisms (Rochalimaea henselae)
iii. Malignant angioendothelioma:
• A malignant intravascular lymphoma and not an angioma or
angiosarcoma

40. Angiosarcomas:
a. Very rare in the lower extremities
b. Very malignant endothelial tumors, usually occurng in the elderly
c. They may be associated with chronic edema
d. They may grow rapidly and may metastasize
e. Most cases are fatal
f. Amputation a good distance proximal to the tumor is the surgical approach
g. Irradiation has not been very effective
h. Some superficial forms (not the deep forms) have been treated
successfully with carbon dioxide laser
i. Patients must be checked for recurrences and metastases

41. Kaposi's Sarcoma (multiple idiopathic hemorrhagic sarcoma):

a. Is related to immunodeficiency, occurs n older patients, AIDS patients, and
patients on chemotherapy or post-transplant immunosuppression therapy
b. May occur n the presence of leukemias and lymphomas
c. Lesions may appear as purpuric macules, papules, patches, nodules,
plaques, and tumors
d. Silent visceral lesions may be present
e. It is presently divided into the following types:
i. Classic (sporadic) epidemic (non AIDS)
• Attributed to the elderly eastern and southern European men
• Skin associated lesions frequently begin on the feet
ii. African- affects adults and children and not restricted to the feet
iii. Allograft-associated (immunosuppression)
iv. Epidemic (AIDS-associated)

NOTE* On occasion, malignant lower extremity tumors may develop that

cannot be clearly identified as to tissue of origin. These include the
alveolar soft-part sarcoma, the mesenchymoma, and any
undifferentiated malignant soft tissue tumor
42. Nodules of Metastatic Lymphoma (non-Hodgkins) or Myeloid
Leukemia (granulocytic sarcoma, chloroma):
a. May occur n the dermis as metastatic site to feet
b. Biopsy is diagnostic
c. Mycosis fungoides is a T-cell lymphoma originating n the skin
i. Woringer-Kolopp disease: Is the localized epidermotropic form formerly
called Pagetoid reticulosis
ii. Sezary syndrome: is the systemic form
• Circulating abnormal lymphoid cells
• Peripheral lymphadenopathy
• Erythroderma
• Intense pruritus

43: Mastocytomas:
a. Are tumors formed by mast cells
b. Some localize to the dermis others are systemic
c. Dermal lesions urticate (Darier's sign)
d. Pruritis and flushing may occur due to local vasodilatory effects of mastcell
derived histamine shock
e. A single local lesion is known as urticaria pigmentosa

44. Metastatic Cancers:

a. Metastatic cancers from visceral organs may occur in the dermis
b. They are usually rapidly growing, multiple (various sites) and otherwise
nonspecific clinically
c. A history if malignant tumor (even years ago) is a clue, but not always
present
d. Even with such a history, a new lesion may be an independent entity