Malignant Mesenchymal Tumors

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Malignant Mesenchymal Tumors
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Fibrosarcoma

• Malignancy of fibroblastic differentiation
• Usually affects adults
• May present as a soft tissue mass or as an
  intrabony lesion

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Fibrosarcoma

• Wide to radical surgical excision
• Like many soft tissue sarcomas, little response
  to radiation or chemotherapy
• 50 five-year survival
• Metastasizes to lung, bone, liver

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Malignant Peripheral Nerve Sheath Tumor

• Also known as neurogenic sarcoma or
  neurofibrosarcoma
• May arise spontaneously or in association with
  neurofibromatosis

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Malignant Peripheral Nerve Sheath Tumor

• Invasive cellular proliferation of spindle-shaped
  cells with wavy nuclei
• Similar appearance with routine hematoxylin and
  eosin-stained sections to several other tumors
• Typically MPNST shows S-100 positivity

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Malignant Peripheral Nerve Sheath Tumor

• Treatment consists of radical surgical excision
• With sporadic cases (not associated with
  NFtosis) 50 five-year survival
• With cases developing in the setting of NFtosis
  10 five-year survival

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Kaposi Sarcoma

• First described by Moritz Kaposi in the late 19th
  century as a disease affecting elderly white
  males of Mediterranean descent
• Subsequently identified in sub-Saharan Africans,
  transplant recipients and HIV males
• Most investigators feel that HHV-8 plays a
  causative role

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Kaposi Sarcoma

• The so-called classic form of KS usually
  appears on the lower extremities
• Initially purple macules develop into plaques
  and, eventually, tumors

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Kaposi Sarcoma

• African form of KS usually involves the lymph
  nodes (lymphadenopathic KS)
• Affects a wide age range of patients, including
  children

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Kaposi Sarcoma

• Transplant-associated KS usually affects the skin
  of a small percentage (1-2) of these patients
• May affect the oral mucosa
• Reducing the degree of immune suppression may
  cause regression of KS

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Kaposi Sarcoma

• AIDS-related KS overwhelming affects gay or
  bisexual men
• Rare examples have been seen in women
• Frequency has been declining, from 25 at onset
  of the epidemic to 10 today
• Of the patients who develop AIDS-KS, 50 will
  have oral lesions

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Kaposi Sarcoma

• Management may include excision for small
  cosmetically unacceptable lesions
• Radiation therapy
• Injections with vinblastine, a cancer
  chemotherapeutic drug

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Kaposi Sarcoma - Prognosis

• Classic fair. Patients usually die of
  something else (MI, CVA, etc.)
• Lymphadenopathic poor. Very aggressive course
• Transplant-associated fair-poor. May regress
  with decreased immune suppression
• AIDS-related fair.

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Rhabdomyosarcoma

• Relatively rare neoplasm of skeletal muscle
  differentiation
• Usually affects children or adolescents
• Head and neck is a common site of involvement
• Infiltrative, rapidly growing mass

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Rhabdomyosarcoma

• When rhabdomyosarcoma grows into a body cavity,
  such as the oral cavity or the vagina, the lesion
  develops an appearance similar to a bunch of
  grapes, thus the term sarcoma botryoides

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Rhabdomyosarcoma

• Treated with multiple modalities
• Wide surgical excision combined with radiation
  therapy and chemotherapy
• Prognosis depends on the stage
• Much improved outlook compared with 30 years ago

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Leukemia

• Group of hematologic malignancies characterized
  by tumor cells circulating in the blood
• Broadly divided into lymphocytic and
  myelomonocytic types
• Further divided into acute and chronic forms

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Leukemia

• Examples would include
• Acute lymphocytic leukemia
• Chronic lymphocytic leukemia
• Acute myelomonocytic leukemia
• Chronic myelomonocytic leukemia

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Leukemia

• Patients often present with signs and symptoms
  related to myelophthisic anemia (normal bone
  marrow cells replaced by leukemic cells)
• Fatigue, SOB, pallor (decreased RBCs)
• Easy bruising (decreased platelets)
• Infection (decreased WBCs)

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Leukemia

• Oral involvement is seen most frequently in the
  myelomonocytic forms of leukemia
• Focal proliferation of leukemic cells at one soft
  tissue site may be seen granulocytic sarcoma
• Diffuse gingival enlargement

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Leukemia

• This diagnosis is usually based on finding
  increased numbers of atypical WBCs in the
  circulation, and the type of leukemia is
  determined by immunohistochemical and cytogenetic
  studies
• If the gingival enlargement caused by leukemia is
  biopsied, an infiltrate of atypical cells is seen

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Leukemia -Treatment

• Chemotherapy
• Bone marrow or stem cell transplantation
• Targeted gene therapy experimental, but
  promising

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Lymphoma

• Malignancy of lymphoid cells
• Usually divided into Hodgkin and non-Hodgkin
  forms
• Hodgkin lymphoma develops in the lymph nodes
• Non-Hodgkin lymphoma may develop in nodes, soft
  tissue or bone

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Hodgkin Lymphoma

• Usually affects teenagers and young adults
  7,600 cases in US in 2003
• Head and neck is common site of initial
  involvement
• One or more rubbery-firm, enlarging, non-tender
  lymph nodes

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Non-Hodgkin Lymphoma

• Tends to affect an older age group than Hodgkin
  lymphoma, and is much more common 53,400 cases
  in US in 2003
• Most present in the lymph nodes, but 30-40 are
  extranodal

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Non-Hodgkin Lymphoma

• Oral involvement of soft tissue is usually seen
  as a diffuse mass of the soft palate or the
  buccal vestibule
• Mandibular involvement may be associated with
  numb chin sign
• Moth-eaten or ill-defined radiolucency seen
  radiographically

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Lymphoma - Treatment

• Localized disease radiation therapy, with or
  without chemotherapy
• More generalized disease - chemotherapy

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Lymphoma - Prognosis

• Hodgkin lymphoma good, with many series
  reporting a 95 10-year survival rate
• Non-Hodgkin lymphoma has a much more variable
  prognosis, depending on the grade and stage of
  disease

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Lymphoma - Prognosis

• Hodgkin lymphoma 1,300 deaths in 2003
• Non-Hodgkin lymphoma 23,400 deaths in 2003