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Benz NapoliMarcella ApriliaYonathan Djaja
   A dermatofibroma is a common overgrowth of the    fibrous tissue situated in the dermis   Also known as : ‘superficia...
   Clinical examination:    ◦ Inspection:      Solitair nodule      overlying skin can range from flesh to gray, yellow...
   Variable frequency USG    ◦ Hypoechoic solid nodule
   No treatment is usually      Indication    necessary                     ◦ Cosmetics   Reassurance that the         ...
   Treatment:    ◦ Do not require treatment    ◦ Occasionally removed in cases of diagnostic      uncertainty, and in som...
   An acrochordon is a small, soft, usually    pedunculated outgrowth of epidermal and dermal    tissue most commonly see...
   Inspection:    ◦   Skin coloured, may be hyperpigmented.    ◦   Solitair or multiple    ◦   Found in intertriginous ar...
   Small, pedunculated acrochordons may be    removed with curved or serrated blade    scissors, while larger skin tags m...
   Rare, slow growing tumour of chondroblastic    derivation.   CMF are firm, grayish white masses that are    sharply d...
   Epidemiology:    ◦ < 1% of primary bone tumours (rare)    ◦ Primarily affects young adults in their second and      th...
   Physical examination:    ◦ 89% involve the lower extremity.    ◦ Proximal tibia is the most common location,      foll...
   Xray : ‘bite’ out of    the metaphyseal    cortex (a diagnostic    feature).    ◦ Well defined    ◦ Elongated    ◦ Rad...
   CMFs are lobulated or    pseudolobulated.   Peripheral condensation    of more cellular tissue    within the lobules....
   Medical care              Surgical care    ◦ No medical care is       ◦ Intralesional      usually necessary         ...
   Definition: Osteofibrous dysplasia (also    known as ossifying fibroma) is a rare, benign    non-neoplastic condition ...
   Most common benign bone tumor in children   Definition: Nonossifying fibromas are made    of fibrous tissue & are typ...
   Definition: (also known as "Desmoplastic    fibroblastoma") is a slow-growing, deep-set,    benign fibrous tumor, usua...
   Definition: is an extremely rare benign tumor that    progresses slowly with well-differentiated cells that    produce...
   Sering terjadi pada anak usia 14 – 18 tahun   Jarang terjadi apabila usia sudah diatas 25    tahun
   Anak    –   anak    yang    terkena   dapat    memunculkan gejala yang berbeda – beda   Biasanya   muncul     berdasa...
   Beberapa gejala yang sering muncul :    ◦ Periodic nosebleed    ◦ Bengkak pada wajah    ◦ Hidung Tersumbat    ◦ Hypona...
   CT – scan  exact location and extension of    the tumor into the skull base   MRI  detailed pictures of organs and ...
   Surgical  untuk di lakukan pengangkatan    tumor   Radioterapi  apabila tumor sudah mencapai    cranium
   Ada beberapa yang menghilang sendiri dan    beberapa ada juga yang tetap bertumbuh.   Ada beberapa tumor yang dapat t...
   Stage IA  Tumor terbatas pada nares    posterior dan/atau nasofaring   Stage IB  Tumor melibatkan nares posterior  ...
   Stage IIIA  Mengerosi dasar tengkorak;    perluasan intrakranial yang minimal.   Stage IIIB  Perluasan ke intrakran...
   Stage I  Tumor terbatas pada kavum nasi,    nasofaring tanpa destruksi tulang.   Stage II  Tumor menginvasi fossa  ...
 benign  tumor that presents as a  small subcutaneous nodule that  slowly increases in size The tumor often has a multi ...
   Most common between 20-50 years old   Slowly growth   40% recurrent   Most on wrist, hands, fingers   Rarely over ...
   Cystic changes in desmoplasmic fibroma of    bone
   Definition: a tumor composed mainly of    fibrous or fully developed connective tissue    that has undergone cystic de...
   noncancerous growths of the uterus that often appear during    your childbearing years.   Also called fibromyomas, le...
Symptoms: Heavy menstrual bleeding Prolonged menstrual periods — 7 days /  more of menstrual bleeding Pelvic pressure /...
Causes Genetic alterations. Hormones. Estrogen & progesterone, two  hormones that stimulate development of the  uterine ...
   Heredity. mother or sister had fibroids   Race. Black women   Pregnancy and childbirth. have a protective effect and...
Histopathology showing poorlydemarcated, dense collagen bundleswith sparsely scattered fibroblasts (A,B).There were also s...
   Definition: rare benign proliferation involving    the dermis & subcutaneous tissues, that is a    collection of dense...
   asymptomatic   long history of a solitary, superficial mass.   The mass is usually in the neck (hence the    name "n...
   pleomorphic fibroma. Cell-depleted, fibrous    connective tissue with atypical fibroblast
Clinical Findings present in adult patients as a slow growing, dome-  shaped papule. The extremities are the most  common...
   Definition: Is a sex cord- stromal tumor. Most    frequently, they occur during middle age, and    rare in children. ...
   Neurofibromatosis: is a genetic disease in    which patients develop multiple soft tumors    (neurofibromas). These tu...
Schwann cell: a type of large cell whose cell membrane wraps spiral myelin sheath
   two types of neurofibromatosis.       Type 1 (von Recklinghausens disease)    ◦ affects 1 in 3000 people;    ◦ the maj...
   Neurofibromatosis 1 (NF1)    gene is located on chromosome 17.    (Normally, this gene produces a protein called    ne...
   The spectrum of lesions in von    Recklinghausens disease varies widely. For    example, the neurofibromas may involve...
   Neurofibromatosis 1:The diagnosis requires 2 of    the following:    ◦    6 discolorations on the skin, 5mm in greates...
   Neurofibromatosis 2: The diagnosis requires    either of the following:    ◦ Eighth cranial nerve masses on both sides...
   depends upon the symptoms. They do not typically need    to be surgically removed.   Surgical exploration and excisio...
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Fibroma

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Fibroma

  1. 1. Benz NapoliMarcella ApriliaYonathan Djaja
  2. 2.  A dermatofibroma is a common overgrowth of the fibrous tissue situated in the dermis Also known as : ‘superficial benign fibrous Histiocytoma’ History of illness ◦ Dermatofibromas typically arise slowly and most often occur as a solitary nodule on an extremity, particularly the lower leg, but any cutaneous site is possible. ◦ Dermatofibromas are usually asymptomatic, but itching and pain often are noted ◦ Although cases of unusually rapid growth exist, most dermatofibromas remain static for decades or persist indefinitely ◦ Woman > man ◦ Young adults most commonly affected.
  3. 3.  Clinical examination: ◦ Inspection:  Solitair nodule  overlying skin can range from flesh to gray, yellow, orange, pink, red, purple, blue, brown, or black. (commonly pink) ◦ Palpation  Usually 0.5- to 1-cm nodule, slightly elevated.  Firm nodule  Fixed to skin surface and is freely movable over the subcutis.  May have tenderness  Dimple Sign on pinching.
  4. 4.  Variable frequency USG ◦ Hypoechoic solid nodule
  5. 5.  No treatment is usually  Indication necessary ◦ Cosmetics Reassurance that the ◦ Symptomatic lesion is benign ◦ Diagnostic uncertainty.  Surgical excision Intralesional steroid  cryosurgery injection has been reported with variable results.Medical care Surgical Care
  6. 6.  Treatment: ◦ Do not require treatment ◦ Occasionally removed in cases of diagnostic uncertainty, and in some patients who request removal as a result of the lesion feeling uncomfortable. ◦ Treatment is by surgical excision.
  7. 7.  An acrochordon is a small, soft, usually pedunculated outgrowth of epidermal and dermal tissue most commonly seen in adults on areas of friction such as the neck, eyelid, axilla, and groin. A.k.a : ‘skin tags’, ‘fibroepithelial polyp (FEP)’ History of illness: ◦ Usually asymptomatic, not painful unless inflammed or irritated ◦ Multiple skin tags are often linked with type 2 DM and obesity. ◦ Statistically significant relationship with obesity. ◦ Usually found in intertriginous area.
  8. 8.  Inspection: ◦ Skin coloured, may be hyperpigmented. ◦ Solitair or multiple ◦ Found in intertriginous area ◦ Pedunculated ◦ Lack of appendages (hair, sweat gland, etc) ◦ Size vary from 2-5 mm, larger acrochordon may sized up to 5cm in diameter. Palpation ◦ Painless ◦ soft
  9. 9.  Small, pedunculated acrochordons may be removed with curved or serrated blade scissors, while larger skin tags may simply require excision. Cryotheraphy Ligation with suture or a copper wire.
  10. 10.  Rare, slow growing tumour of chondroblastic derivation. CMF are firm, grayish white masses that are sharply demarcated; they are lobulated or pseudolobulated. Their appearance can mimic fibrous tissue or hyaline cartilage. Many display morphologic features that resembles different stage of chondrogenesis.
  11. 11.  Epidemiology: ◦ < 1% of primary bone tumours (rare) ◦ Primarily affects young adults in their second and third decades of life. ◦ 80% of patients <36y.o. Symptoms: ◦ Pain is the most common symptom (may present for years). Typically mild pain, but may proggressively becoming more severe. ◦ Rarely limitation of joint motion.
  12. 12.  Physical examination: ◦ 89% involve the lower extremity. ◦ Proximal tibia is the most common location, followed by distal femur, pelvis, and foot. ◦ Usually in long bones. ◦ Flat bone involvement may be observed more often in older adults. Lab studies ◦ Usually does not show any laboratory abnormalities.
  13. 13.  Xray : ‘bite’ out of the metaphyseal cortex (a diagnostic feature). ◦ Well defined ◦ Elongated ◦ Radioluscent ◦ Greatest dimension is typically 1-10cm. ◦ Usually occur in the metaphysis of long bones. ◦ Matrix calcifications are unusual.
  14. 14.  CMFs are lobulated or pseudolobulated. Peripheral condensation of more cellular tissue within the lobules. Composed of myxoid or chondroid tissue. The centre of each lobule is hypocellular. Surrounding stroma is denser, with spindle shaped cells, blood vessels and occasional multinucleate giant cells.
  15. 15.  Medical care  Surgical care ◦ No medical care is ◦ Intralesional usually necessary curretage ◦ NSAID or other (recurrence rates analgetic for pain approx 25% with control curretage and bone graft) ◦ En bloc excision (lower recurrence rate)
  16. 16.  Definition: Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause
  17. 17.  Most common benign bone tumor in children Definition: Nonossifying fibromas are made of fibrous tissue & are typically yellow / brown in color. They most commonly occur in the flared end of the lower thighbone (femur) and in the flared upper or lower ends of the shinbone (tibia). NOFs sometimes occur in the smaller bone in the lower leg (fibula), and in the upper arm bone (humerus).
  18. 18.  Definition: (also known as "Desmoplastic fibroblastoma") is a slow-growing, deep-set, benign fibrous tumor, usually located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions.
  19. 19.  Definition: is an extremely rare benign tumor that progresses slowly with well-differentiated cells that produce collagen. The most common site is 1.the mandible 2.femur 3. pelvis. It occurs most often in the first 3 decades found equally in men & women. Clinical findings: pain late in the clinical course & swelling. The diagnosis of desmoplastic fibroma is difficult to make radiologically. Plain xray shows an osteolytic, expansile, medullary lesion with well defined sclerotic margins.
  20. 20.  Sering terjadi pada anak usia 14 – 18 tahun Jarang terjadi apabila usia sudah diatas 25 tahun
  21. 21.  Anak – anak yang terkena dapat memunculkan gejala yang berbeda – beda Biasanya muncul berdasarkan dimana angiofibroma ini muncul
  22. 22.  Beberapa gejala yang sering muncul : ◦ Periodic nosebleed ◦ Bengkak pada wajah ◦ Hidung Tersumbat ◦ Hyponasal speech (due to not enough air flow through the nose) ◦ Otorrhea (Draining ear)
  23. 23.  CT – scan  exact location and extension of the tumor into the skull base MRI  detailed pictures of organs and structures within the bodyThese tests allow your child’s surgeon to accurately determine how far the lesion has spread and to plan the surgical approach.
  24. 24.  Surgical  untuk di lakukan pengangkatan tumor Radioterapi  apabila tumor sudah mencapai cranium
  25. 25.  Ada beberapa yang menghilang sendiri dan beberapa ada juga yang tetap bertumbuh. Ada beberapa tumor yang dapat tumbuh kembali setelah dilakukan pembedahan. Prompt medical attention and aggressive therapy are important for the best prognosis and timely treatment.
  26. 26.  Stage IA  Tumor terbatas pada nares posterior dan/atau nasofaring Stage IB  Tumor melibatkan nares posterior dan/atau nasofaring dengan perluasan ke satu sinus paranasal Stage IIA  Perluasan lateral minimal ke dalam fossa pterygomaxila Stage IIB  Mengisi seluruh fossa pterygomaksila dengan atau tanpa erosi ke tulang orbita.
  27. 27.  Stage IIIA  Mengerosi dasar tengkorak; perluasan intrakranial yang minimal. Stage IIIB  Perluasan ke intrakranial dengan atau tanpa perluasan ke dalam sinus kavernosus
  28. 28.  Stage I  Tumor terbatas pada kavum nasi, nasofaring tanpa destruksi tulang. Stage II  Tumor menginvasi fossa pterygomaksila, sinus paranasal dengan destruksi tulang. Stage III  Tumor menginvasi fossa infra temporal, orbita dan/atau daerah parasellar sampai sinus kavernosus. Stage IV  Tumor menginvasi sinus kavernosus, chiasma optikum dan/atau fossa pituitary.
  29. 29.  benign tumor that presents as a small subcutaneous nodule that slowly increases in size The tumor often has a multi nodular growth pattern
  30. 30.  Most common between 20-50 years old Slowly growth 40% recurrent Most on wrist, hands, fingers Rarely over 2 cm, never over 6 cm
  31. 31.  Cystic changes in desmoplasmic fibroma of bone
  32. 32.  Definition: a tumor composed mainly of fibrous or fully developed connective tissue that has undergone cystic degeneration
  33. 33.  noncancerous growths of the uterus that often appear during your childbearing years. Also called fibromyomas, leiomyomas / myomas, uterine fibroids arent associated with an increased risk of uterine cancer & almost never develop into cancer.
  34. 34. Symptoms: Heavy menstrual bleeding Prolonged menstrual periods — 7 days / more of menstrual bleeding Pelvic pressure / pain Frequent urination Difficulty emptying bladder Constipation Backache / leg pains
  35. 35. Causes Genetic alterations. Hormones. Estrogen & progesterone, two hormones that stimulate development of the uterine lining during each menstrual cycle in preparation for pregnancy, appear to promote the growth of fibroids. Fibroids contain more estrogen and progesterone receptors than do normal uterine muscle cells. Other chemicals. insulin-like growth factor, may affect fibroid growth
  36. 36.  Heredity. mother or sister had fibroids Race. Black women Pregnancy and childbirth. have a protective effect and may decrease risk of developing uterine fibroids. Areas of research Research examining other potential factors in the development of fibroids continues in these areas: Obesity. obese women Oral contraceptives. women who take oral contraceptives have a lower risk of fibroids. except those who start oral contraceptives between ages 13 and 16. progestin-only contraceptives may decrease risk
  37. 37. Histopathology showing poorlydemarcated, dense collagen bundleswith sparsely scattered fibroblasts (A,B).There were also some interspersedadipose tissues (C),and entrapped nerve fibers (D)
  38. 38.  Definition: rare benign proliferation involving the dermis & subcutaneous tissues, that is a collection of dense, hypocellular bundles of collagen with entrapped adipocytes & increased numbers of small nerves. It is no longer called a nuchal fibroma, but instead a "nuchal-type fibroma" since it develops in other anatomic sites
  39. 39.  asymptomatic long history of a solitary, superficial mass. The mass is usually in the neck (hence the name "nuchal-type"), but it can be seen in the extremities, lumbosacral area, buttocks, and face. There is a strong association with diabetes mellitus and Gardner syndrome; in fact, it may be the initial manifestation of Gardner syndrome.
  40. 40.  pleomorphic fibroma. Cell-depleted, fibrous connective tissue with atypical fibroblast
  41. 41. Clinical Findings present in adult patients as a slow growing, dome- shaped papule. The extremities are the most common location followed by the trunk, head and neck. Clinically they can resemble a skin tag, nevus, or neurofibroma.Histologic Features The tumors are well-circumscribed, paucicellular lesions composed of widely scattered spindled, stellate & pleomorphic multinucleated giant cells . Mitotic figures are rare and atypical mitotic figures are almost never seen. The stroma is usually densely collagenous but myxoid change may be seen and rarely is a prominent feature. Adnexal structures are absent.
  42. 42.  Definition: Is a sex cord- stromal tumor. Most frequently, they occur during middle age, and rare in children. On gross pathology, they are firm and white or tan. On microscopic examination, there are intersecting bundles of spindle cells producing collagen.
  43. 43.  Neurofibromatosis: is a genetic disease in which patients develop multiple soft tumors (neurofibromas). These tumors occur under the skin and throughout the nervous system. Neurofibroma: A tumor of peripheral nerves due to abnormal proliferation of Schwann cells, called also fibroneuroma
  44. 44. Schwann cell: a type of large cell whose cell membrane wraps spiral myelin sheath
  45. 45.  two types of neurofibromatosis. Type 1 (von Recklinghausens disease) ◦ affects 1 in 3000 people; ◦ the majority of cases are mild with a limited number of tumors. Type 2 affects 1 in 50,000 people
  46. 46.  Neurofibromatosis 1 (NF1) gene is located on chromosome 17. (Normally, this gene produces a protein called neurofibromin, which is abundant in nervous system tissue and helps regulate cell growth. ) A mutation of the NF1 gene causes a loss of neurofibromin, allows cells to grow uncontrolled. Neurofibromatosis 2 (NF2) gene is located on chromosome 22 produces a protein called merlin. A mutation of the NF2 gene causes loss of merlin, which also leads to uncontrolled cell growth
  47. 47.  The spectrum of lesions in von Recklinghausens disease varies widely. For example, the neurofibromas may involve small skin nerves and appear as small bumps, or they may be confined to large nerves and produce elephantiasis, or enlargement of affected body parts.
  48. 48.  Neurofibromatosis 1:The diagnosis requires 2 of the following: ◦ 6 discolorations on the skin, 5mm in greatest diameter in pre-adolescents & greater than 15 mm in adolescents and adults. ◦ 2 neurofibromas. ◦ Freckling in the arm pits or groin regions. ◦ Optic glioma. ◦ Two or more Lisch nodules / small masses on the iris of the eyes. ◦ Bone lesions, called sphenoid dysplasia. ◦ A first degree relative with neurofibromatosis type 1.
  49. 49.  Neurofibromatosis 2: The diagnosis requires either of the following: ◦ Eighth cranial nerve masses on both sides (bilateral). ◦ An immediate relative with neurofibromatosis 2, and either an eighth nerve mass on one side or two of the following: neurofibroma, meningioma, glioma,or schwannoma.
  50. 50.  depends upon the symptoms. They do not typically need to be surgically removed. Surgical exploration and excision may be required under the following circumstances: -type of diagnosis -pain -cosmetic considerations -progressive neurological complications -compression of adjacent tissues -suspicion of a malignant tumor. Painful neurofibromas occur just under the skin. These globular lesions may occur in the hand or other surfaces that are subject to pressure. Typically these lesions involve small bundles of superficial nerves and can be removed without creating a meaningful neurological deficit.

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