Malignant Mesenchymal Neoplasms Of Soft Tissue PDF

MALIGNANT ORAL SOFT TISSUE NEOPLASMS Dr Divya Gopinath BDS,MDS,MPhil,PhD DIVYA GOPINATH DIVYA GOPINATH Soft tissue sarcomas Rare in oral and maxillofacial region 40 years; 5% of all cases Extremities Painless mass- grow rapi...

MALIGNANT ORAL SOFT TISSUE NEOPLASMS Dr Divya Gopinath BDS,MDS,MPhil,PhD DIVYA GOPINATH DIVYA GOPINATH Soft tissue sarcomas Rare in oral and maxillofacial region 40 years; 5% of all cases Extremities Painless mass- grow rapidly Face and orbit> Nasal cavity Intraoral- Palate DIVYA GOPINATH  Embryonal rhabdomyosarcoma ◦ Alternating hypercellular areas and hypocellular areas with myxoid collagenous or stroma ◦ Densely cellular subepithelial zone (cambium layer) composed of primitive, small cells with hyperchromatic nuclei and mitotic activity ◦ Rhabdomyoblasts typically seen in hypocellular foci DIVYA GOPINATH  It resembles various stages in the embryogenesis of skeletal muscle. Rhabdomyoblasts: Plump cells with Eccentric nuclei, dense eosinophilic cytoplasm Elongated strap cells with eosinophilic cytoplasm and cross striations DIVYA GOPINATH  Elongated strap cells with eosinophilic cytoplasm and tadpole cells (unipolar) cross striations DIVYA GOPINATH Alveolar rhabdomyosarcoma ◦ Nests and sheets of primitive small round cells with abundant eosinophilic cytoplasm ◦ Noncohesive cells floating in empty spaces create characteristic alveolar pattern DIVYA GOPINATH Pleomorphic rhabdomyosarcoma Sheet-like growth of pleomorphic round to spindled rhabdomyoblasts DIVYA GOPINATH Treatment and prognosis Treatment typically consists of local surgical excision followed by multiagent chemotherapy (vincristine, actinomycin D, and cyclophosphamide). 5 year survival ◦ Embryonal NOS- 66-72% ◦ Botryoid (type of embryonal)- 95% ◦ Alveolar- 44-53% DIVYA GOPINATH DIVYA GOPINATH Lymphoma The main difference between leukemia and lymphoma is that leukemia affects the blood and bone marrow, while lymphomas mainly affect the lymph nodes. Two types Hodgkins lymphoma and Non hodgkins lymphoma Hodgkins lymphoma Hodgkin lymphoma almost always begins in the lymph nodes, and any lymph node group is susceptible. The most common sites of initial presentation are the cervical and supraclavicular nodes. Overall, a male predilection is observed, and a bimodal pattern is noted (One peak between 15 and 35 years of age; another peak is seen after the age of 50). DIVYA GOPINATH The usual presents enlarging, nontender, discrete masses in one lymph node region. If it is untreated, then the condition spreads to other lymph node groups and eventually involves the spleen and other extralymphatic tissues, such as bone, liver, and lung. Oral involvement is rare. In about 30% to 40% of patients with Hodgkin disease, other systemic signs and symptoms may be present, such as weight loss, fever, night sweats, and generalized pruritus (itching). The absence of these systemic signs and symptoms is considered to be better in terms of the patient's prognosis, DIVYA GOPINATH Histopathology Normal nodal architecture is destroyed by diffuse, infiltrate of large, atypical neoplastic lymphoid cells This atypical cell is known as a Reed-Sternberg cell. The Reed- Sternberg cell is typically binucleated (“owl- eye” nuclei), although it may be multinucleated (“pennies on a plate”), with prominent nucleoli. Treatment Radiotherapy and chemotherapy DIVYA GOPINATH Non Hodgkins lymphoma (NHL), Classified mainly as B cell lymphomas (more common) and T cell lymphomas The condition most commonly develops in the Lymph nodes, 40% to 50% of lymphomas develop in an extranodal site, oral cavity is an extranodal site Histopathology Proliferation of lymphocytic-appearing cells that may show varying degrees of differentiation, depending on the type of lymphoma. DIVYA GOPINATH Oral manifestations (NHL) The malignancy may develop in the oral soft tissues or centrally within the jaws. Soft tissue lesions appear as nontender, diffuse swellings; affect the buccal vestibule, posterior hard palate, or gingiva The lesion may appear erythematous or purplish, and it may or may not be ulcerated. DIVYA GOPINATH Burkitt lymphoma A type of non hodgkin lymphoma of B-lymphocyte origin that represents an undifferentiated lymphoma. African ethnicity EBV is considered to be an etiological agent Predilection for the jaws. Affects children Histopathology “starry-sky” pattern is often appreciated—a phenomenon that is caused by the presence of macrophages in between sheets of atypical lymphocytes DIVYA GOPINATH Leukemia Leukemia is a heterogeneous group of hematologic malignancies that arise from the dysfunctional proliferation of immature eukocytes The disease begins with the malignant transformation of one of the hematopoietic stem cells, which initially proliferates in the bone marrow and eventually overflows into the peripheral blood of the affected patient. According to clinical course Acute Chronic According to origin Lymphoid- formed of lymphocytes Myeloid-granulocytes or monocytes, and less frequently, erythrocytes or megakaryocytes. DIVYA GOPINATH Clinical features Acute leukemia tends to present non-specifically, although the most common presenting features include fever, lethargy, and bleeding. Hepatosplenomegaly, lymphadenopathy, and musculoskeletal symptoms can also be clues to the diagnosis. It may affect anyone at any age, but children younger than 15 and adults older than 50 are more likely to develop the condition. Chronic leukemia subtypes occur almost exclusively in adults. Many patients are asymptomatic at the time of diagnosis, identified only incidentally after marked leukocytosis is discovered on a complete blood count (CBC) performed for another reason. DIVYA GOPINATH  Oral manifestations Oral manifestations are more common in may occur in any type of leukemia, but they are more prevalent in acute (vs chronic) and myeloid (vs lymphoid) leukemias 1. Ulceration of the oral mucosa -as a result of the impaired ability of the host to combat the normal microbial flora. Gingival mucosa is the most severely affected because of the abundant bacteria normally present around the teeth. The ulcers that are produced are typically deep,punched- out lesions with a gray-white necrotic base. DIVYA GOPINATH 2. Gingival enlargements Leukemic cells infiltrate the oral soft tissues and produce a diffuse, boggy, nontender swelling that may or may not be ulcerated. This occurs most frequently with the myelomonocytic types of acute leukemia, and it may result in diffuse gingival enlargement Microscopic examination of leukemia-affected tissue shows diffuse infiltration and destruction of the normal host tissue by sheets of poorly differentiated round cells DIVYA GOPINATH 3. Repeated infections Patients with leukemia may also develop recurrent viral, bacterial and fungal infections, like herpes or candidiasis, as a consequence of immunosuppression 4. Bleeding manifestations Gingival oozing, petechiae, hematomas, or ecchymoses DIVYA GOPINATH Diagnosis and Treatment The diagnosis is usually established by confirming the presence of poorly differentiated leukemic cells in the peripheral blood and bone marrow. Effective early diagnosis is indispensable for survival. Therefore, dentists are responsible for early detection of oral manifestations of leukemia and for a fast referral to a hematologist. The treatment of a patient with leukemia consists of various forms of chemotherapy; the type of leukemia dictates the chemotherapeutic regimen DIVYA GOPINATH Metastasis to oral cavity Uncommon-1% of all oral malignancies Soft tissues Bone BATSON PLEXUS: valveless vertebral venous plexus that might allow retrograde spread of tumor cells, bypassing filtration through the lungs DIVYA GOPINATH C/F SOFT TISSUE: gingiva (54%) followed by tongue (22.5%) ◦ Resemble hyperplastic tissue/ pyogenic granuloma ◦ Tooth loosened- underlying bone destruction ◦ Most metastasis tend to deposit on the rich vascular network of inflamed gingival tissue- as it is fertile site for metastatic deposits ◦ Male>females ◦ Middle and older adults ◦ Lung>RCC>melanoma-men ◦ Breast ca- women ◦ In 25% of cases, oral lesion is the first sign of metastasis DIVYA GOPINATH DIVYA GOPINATH BONE ◦ 43-52 years ◦ No gender predilection ◦ Posterior mandible ◦ Pain, swelling, mobile teeth, parasthesia ◦ Involvement of mental nerve: NUMB CHIN SYNDROME ◦ Breast, lung, thyroid, prostrate and kidney ◦ Radiographically: ◦ mostly ill-defined radiolucencies/ moth eaten appearance ◦ Some are osteoblastic- prostrate and breast carcinomas DIVYA GOPINATH DIVYA GOPINATH Treatment and prognosis Very poor prognosis PALLIATIVE DIVYA GOPINATH THANK YOU DIVYA GOPINATH