Topic include all categories of fibromatsis

2. DR ARUN HALDIA

3. INTRODUCTION
 Comprise a group of benign fibrous tissue proliferations of
similar microscopic appearance whose biologic behavior is
intermediate between that of benign fibrous lesion &
fibrosarcoma
 It is characterised by infiltrative growth, tendency toward
recurrence but never metastasize
 Deep fibromatoses are rarer than their superficial
counterparts and are encountered in two to four
individuals/million population per year

4. CLASSIFICATION
• Superficial (fascial) fibromatosis
• Palmer fibromatosis(dupuytren’s ds)
• Planter fibromatosis ( ledderhose’s ds)
• Penile fibromatosis (peyronie’s ds)
• Knuckle pads
• Deep (musculoaponeurotic ) fibromatosis
• Extraabdominal fibromatosis
• Abdominal fibromatosis
• Intraabdominal fibromatosis
• Pelvic fibromatosis
• Mesentric fibromatosis
• Mesentric fibromatosis in gardner syndrome

5. SUPERFICIAL (FASCIAL)
FIBROMATOSIS
DEEP (MUSCULOAPONEUROTIC)
FIBROMATOSIS
Small size Often large size
Slow growing Rapidly growing
Arise from the fascia or aponeurosis,
Rarely involve deep structures
Arise from deep structures
divided into
1) an early rather cellular proliferative
phase
2) Late: richly collagenous regressive or
contractile
More aggressive
Has high recurrence rate

6. PALMER FIBROMATOSIS
 Also k/a DUPUYTREN’S DISEASE or CONTRACTURE
 MC type of fibromatosis
 Affect adult, with increase in incidence with advancing age. M>F(3-4
times higher). Rare in <30yr
 50% bilateral
 Patient present with flexion contractures
principally affecting the 4th and 5th digits
 Associated Ds.-Planter F(5-20%),
penile F(2-4%), Knuckle pads,
polyfibromatosis syndrome, diabetes mellitus

7.  GROSS: single nodule <1cm or conglomerate of several
nodule.cut surfae- grey yellow to grey white
 MICROSCOPY
Palmer fibromatosis c/o parallel fascicles of slender fibroblast separated by variable
amount of collagenous stroma

8. Uniform fibroblastic
proliferation in palmer
fibromatosis
Cellular form of
palmer F showing
uniform spindled
cells separated by
collagen

9. IHC
Positive for vimentin, and variably for SM Actin & Ms specific Actin
depend upon stage & degree of diff.
D/D
 Fibrosarcoma: rare in hand, deap seated, cells are arranged in
long fasicles or herringbone pattern, show greater degree of
nuclear hyperchromasia, pleomorphism, mitotic activity &
occ. necrosis

10. PLANTER FIBROMATOSIS
 Characterised by nodular fibrous proliferation within
planter aponeurosis
 Incidence increase with age. As compared to Palmer F.
much greater incidence in children and adolescents

11. MICROSCOPY
Planter F showing the
characteristic nodular
growth pattern
Planter F c/o
unifrom
spindle cells
arranged in
long fasicles

12. PENILE FIBROMATOSIS
(PEYRONIE’S DISEASE)
 Superficial form of fibromatosis that result in an ill-
defined fibrous thickening or plaque like mass in the
shaft of the penis
 Seen in men 45-60yr

13. KNUCKLE PADS
 Microscopically it resembles
palmer fibromatosis but digital
contractures do not occur
 Grossly, it may confused with
pad-like hyperkeratosis that
occur sec. to trauma or self
manipulation
 It may precede the onset of
palmer or planter F. & may
disappear spontaneously after
these lesion are excised

14. EXTRAABDOMINAL FIBROMATOSIS
(EXTRA-ABDOMINAL DESMOID)
 Arises principally from the connective tissue of muscle & the
overlying fascia or aponeurosis(musculoapnoneurotic F.)
 The term Aggressive fibromatosis is often employed to emphasize its
frequently aggressive behavior
 Site : shoulder & pelvic girdle and the thigh of adolescent & young
adults
 Age- peak at 25-35yr, F>M
 Pt. present with a deep seated,firm,poorly circumscribed mass with
little or no pain

15. GROSS
 Most tumors
measure 5-10cm
 Firm, cut with
gritty sensation
 Cut surface reveals
a glistening white,
coarsley
trabeculated
surface

16. MICROSCOPY
 Characteristically,
the lesion is
poorly
circumscribed &
infiltrate the
sorrounding
tissue, usually
striated muscle

17. Interlacing bundles of
fibroblasts separated by
variable amounts of
collagen in extra-
abdominal fibromatosis
Extra-abdominal
fibromatosis:
storieform pattern

18. Peripheral portion of
extra-abdominal
fibromatosis with
entrapped muscle giant
cells
High-power view of
extra-abdominal
fibromatosis showing
vesicular nuclei with
minute nucleoli,
rather indistinct
cytoplasm, and
interstitial collagen

19. D/D
 Fibrosarcoma: cells arranged in a more consistent sweeping
fascicular(herringbone) pattern, more uniformly cellular ,
cells are often overlapping & separated by less collagen.
Nuclei are more hyperchromatic, atypical and have
prominent nucleoli. Mitotic count - >1/10hpf
 Reactive fibroblastic proliferation: have a more variable
growth pattern & frequently have focal haemrrhage or
hemosiderin deposit, often situated along blood vessels
 Desmoplastic fibroma(when it present as soft tissue maas):
predominant in metaphyseal or diaphyseal portion of long
bones or in jaw. Radiographic studies are also useful

20.  Myxoma(small Bx or needle BX) : paucicellular with
cells separated by abundant myxoid matrix while
fibromatosis display a more cellularity & more
interstitial collagen than myxoma

21. ABDOMINAL FIBROMATOSIS
(ABDOMINAL DESMOID)
 Similar to extra-abdominal fibromatosis
 Occur in women of childbearing age
during or following pregnancy
 Tumor arise from musculoaponeurotic
structures of the abd wall, esp the rectus
& internal oblique ms & their covering
 Grossly & microscopically appearance
similar to extr-abdominal fibromatosis
except tumor is small & behave less
destructively, also these lesion are
variably cellular & often hypocellular &
c/o cells with normochromatic nucleoli
with pinpoint nucleoli

22. INTRA-ABDOMINAL FIBROMATOSIS
INTRA-ABDOMINAL DESMOID
 Group of closely related lesions distinguished from
one another by the clinical setting & location
 This category includes:
 Pelvic fibromatosis
 Mesentric fibromatosis
 Mesentric fibromatosis in Gardner syndrome

23. PELVIC FIBROMATOSIS
 Site: iliac fossa and lower portion of the pelvis
 Occur chiefly in young women of 20-35yr
 Pt. present with slow growing mass that is
asymptomatic or causes only slight pain
 Often mistaken for an ovarian neoplasm of messentric
cyst
 Grossly & histologically similar to extra-abd or abd
fibromatosis

24. MESENTRIC FIBROMATOSIS
 It is the m.c. primary tumor of the mesentry
 Most cases are sporadic but some are a/w Gardner
syndrome, trauma, or hyperestrogenic states.
 Mean age-35yr
 Site : mesentry of small bowel, ileocolic mesentry,
gastrocolic ligament, omentum, or retroperitoneum
 Most pt. present with asymptomatic abd mass
 Gross : large >10cm, well circumscribed
 Complication may be caused by compression of ureter ,
small or large intestine or development of intestinal
perforation
 Locally recurrent

25. MICROSCOPY
 Composed of cytologically bland spindle shaped or
stellate cells evenly deposited in a densely collagenous
stroma
 Typically there is variable cellularity with some area
show dense fibrous tissue, some may show myxoid
change
 Scattered keloidal type collagen may be present
 There is prominent dilated thin walled blood vessels

26. Uniform spindled proliferation in mesenteric fibromatosis

27. Orderly arrangement of uniform fibroblasts associated with moderate amounts
of collagen and mucoid material in mesenteric fibromatosis

28. Prominent dilated blood vessels with perivascular hyalinization,
a common feature of intra-abdominal fibromatosis

29. Intraabdominal desmoids can extend through the bowel to the M. mucosa

30. This example of desmoid fibromatosis shows a keloidal collagenous stroma and may
evoke keloidal scar

31. MESENTRIC FIBROMATOSIS IN
GARDNER SYNDROME
 In 1951 Gardner reported the familial occurrence of intetinal
polyposis, osteomas, fibromas & epidermal or sebaceous cyst,
the term GARDNER SYNDROME was coined by Smith in 1958
 Autosomal dominant caused by a germline mutation in APC
gene on long arm of chromosome 5
 more common in women of 25-35yr
 Cutaneous cyst are present in 40-50% & osteomas are present in
35-50%. As a rule, the cutaneous cyst & osteomas during
chilhood or teenage years & precede the onset of polyposis &
fibromatosis by 10-15yr
 Intestinal polyp are present throughout the intestinal tract
 Colorectal adenoca occur 10-15yr after onset of polyposis
 This syndrome may be a/w adrenal corical ca, ampulla of vater
ca, & thyroid ca
 Histology is similar to other form of fibromatosis

32. Low-power view of mesenteric fibromatosis in Gardner syndrome showing uniform
fibrocollagenous growth infiltrating the wall of the small bowel

33. IHC
 Positive stain: vimentin, B-catenin, variable smooth
ms actin & muscle sp actin
 Negative stain: CD34, keratin, S-100, desmin, CD117

34. D/D
 Mesentric panniculitis & mesentric
lipodystrophy(sclerosing mesentritis): these condition
are characterised by fibrosis & chronic inflammatory
infiltrate
 fibrosarcoma
 Idiopathic retroperitoneal fibromatosis
 GIST
 Leiomyoma
 Neurofibroma
 Schwannoma
 Keloid

35. SARCOMA vs INTRA ABD FIBROMATOSIS
fibrosarcoma: more cellular, less collagen, atypia, high
proliferative index ,

36. IDIOPATHIC RETROPERITONEAL
FIBROSIS vs INTRA-ABD FIBROMATOSIS
Idiopathic retroperitoneal fibromatosis: more densely hyalinised &
more inflamed

37. GIST vs INTRA-ABD FIBROMATOSIS
GIST: CD117+, CD34+

38. LEIOMYOMA vs INTRA-ABD FIBROMATOSIS
Leiomyoma: bright pink
cytoplasm of smooth muscle
with oblong nuclei, desmin+

39. NEUROFIBROMATOSIS vs INTRA-ABD FIBROMATOSIS
Neurofibroma : no myofibroblasts, S100+

40. SCHWANNOMA vs INTRA-ABD FIBROMATOSIS
Schwannoma: palisading Schwann cells, usually
minimal collagen, S100+

41. KELOID vs INTRA-ABD FIBROMATOSIS
Thick collagen surrounded by paler staining fibroblasts
Clinical history : typically dark skinned individuals, prior trauma, burn or
surgery

42. CASE PRESENTATION
 An 18y male presented in surgery deptt. with c/o pain
abdomen since one month
 On P/A examination they found a 15x15cm mobile,
firm, intra-abdominal lump with normal overlying
skin
 No family h/o of polyposis
 Clinical Dx - ? SARCOMA

43. Single lump ms 21x11x15cm with attached loop of intestine ms 55.0cm in length
with a messentric mass ms 18x10x10cm, circumscribed & partially capsulated.
Intestine is thinned-out over a segment with underlying mass

44. Cut-surface is grey-white with focal area of peripheral haem. No area of
necrosis seen

45. Shows sweeping budles & fasicles of spindle shaped cells with intervening
collagen.

46. Cells varying from thin to plump myofibroblastic type, nuclei are also tapering to ovoid.
Mild AN. Mitosis- negligibe

47. Intralesional haemorrhage

48. Keloidal type collagen

49. Melting insinuation: tumor is merging with muscularis
propria

50. IHC
POSITVE STAIN NEGATIVE STAIN
 B-catenin
 Vimentin
 Desmin
 Smooth ms Actin
 CD117
 CD34
 S-100
 Ki-67: very low

51. DIAGNOSIS
 MESSENTRIC FIBROMATOSIS

52. THANKS