Hypodontia or supernumerary teeth, hypercementosis, and hypoplasia of the mandible and maxilla may also be seen (Fig.В 2). Patients often have a high arched palate with delayed eruption of both deciduous and permanent teeth and persistence of deciduous teeth leading to crowding and increasing vulnerability to dental caries.

A highly obtuse gonial angle of the mandible is pathognomonic of pyknodysostosis, often with prognathism, with higher risk of mandibular fracture and osteomyelitis (Fig.В 2). There are also peculiar mandibular and dental manifestations (Fig.В 2). Patients suffer from dolichocephaly, delayed closure of sutures or persistently open fontanelles (Fig.В 2), multiple wormian bones, hypoplastic paranasal sinuses, beaked appearance of the nose, and frontoparietal bossing. Her WBC count had been reported as normal on several occasions and hence was not pancytopenic, and the pneumonia was likely an incidental finding.Д¬haracteristic features include short stature, craniofacial abnormalities, frequent fractures, and higher risk of osteomyelitis. In the background of pyknodysostosis, she was suspected to have myelophthisic anemia. On clinical examination, she had hepatosplenomegaly, which was felt to represent additional sites of extramedullary hematopoiesis. There was no hemolysis on her blood smear. Her ESR and reticulocyte count were elevated at 46 and 3.1% respectively. Her Vitamin B12 and folate levels were normal. Her iron saturation was 22% and ferritin 98. Her blood smear showed nucleated RBC, metamyelocytes, and myelocytes. Prior records confirmed that these counts had been low for several years. Her most recent hemoglobin (Hgb) was low в€’вЂ‰7.1 and so was her platelet count в€’вЂ‰95,000. On further review of her charts, the patient had a history of long-standing fatigue, for which she was being followed by a hematologist. These findings were consistent with extramedullary hematopoiesis. CT of the neck demonstrated bilateral lobulated paraspinal soft tissue masses left larger than right (Fig.В 1 *). Other characteristic changes noted on CT face included calvarial thickening, non-closure of cranial sutures, underpneumatization of sinuses and mastoid air cells, and diffuse sclerosis of the visualized bones. Plain films of the face and cervical spine revealed an obtuse gonial angle (Fig.В 2), a hallmark feature of this entity. Prior imaging workup performed included plain film radiography and CT of the face and neck. The patient was noted to have a history of pyknodysostosis, multiple related fractures including C1 vertebral body and hearing loss. Incidentally, she was noted to have a lobulated paraspinal mass in her left hemithorax (Fig.В 1). Her chest radiograph demonstrated a right upper lobe consolidation representing pneumonia. Additionally, anti-cancer drugs may be administered to reduce enzyme development that is essential for tumor maturation.The 49-year-old female patient presented to the emergency department with cough and shortness of breath for a few days. Radiation and chemotherapy may be utilized simultaneously to effectively target and eliminate cancerous cells. In severe cases, amputation of the affected extremity may be necessary to prevent the further spread of cancerous cells. Surgery may be the first step in any treatment regimen and involves the removal of the tumor and any surrounding tissue which may be affected. Treatment for this condition is usually dependent on the individual and factors including the cancer's size, location, and staging. A stage four soft tissue sarcoma is the most advanced stage, having aggressively affected the body's lymphatic system, as well as other parts of the body. Sarcomas given a two or three staging are more advanced in nature, larger in size, and may be more aggressive and invasive to surrounding tissues and organs. Stage one cancers are considered small in size and have not affected the surrounding tissue. The extent of a diagnosed cancer is assigned a staging number from one to four.

Diagnosis code soft tissue mass