Total patellectomy for patellar aneurysmal bone cyst.

Herein we report a case of patellar aneurysmal bone cyst of a 32-year-old female patient who was admitted to our outpatient clinic due to the swelling on her left knee. She was describing no trauma history but increasing pain and that the mass was expanding gradually by the time. Although it occurs rarely in patella, evaluation by radiographic and magnetic resonance images revealed that the mass was due to the patellar aneurysmal bone cyst, which also destructed the surface of the joint. Although there was no impairment in the range of motion of the patient's left knee in the preoperative evaluation, because of the destruction, we performed total patellectomy and extensor mechanism reconstruction by Zaricznyj technique instead of curettage and grafting. The patient was satisfied with the procedure performed after the surgery and there was no complication in the follow-up.

An aggressive and lethal course of Churg-Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy.

Churg-Strauss syndrome (CSS) is a rare type of necrotizing vasculitis affecting small to medium-sized vessels typically characterized by asthma, lung infiltrates, necrotizing granulomas and hypereosinophilia. Herein, we describe a case of CSS presenting severe and aggressive course. A 35-year-old male patient with weight loss, dyspepsia, dyspnea and hemoptysis was admitted. The laboratory analyses indicated a remarkable eosinophilia, elevated levels of serum total IgE and positive cANCA. Thorax CT findings were suggestive of alveolar hemorrhage. Bronchoalveolar lavage revealed alveolar hemorrhage with eosinophilia and transbronchial lung biopsy showed eosinophilic vasculitis. Cardiac enzymes were increased and murmurs were audible revealing cardiomyopathy proven by echocardiography. Pulse cyclophosphamide and methyl prednisolone was immediately started. On the 21st day, intestinal perforation developed and urgent surgery was performed. During a follow-up, although a radiological improvement was observed in the chest X-ray, cardiac failure, peripheral neuropathy and skin lesions developed and high-dose intravenous immunoglobulin and anti-TNF therapy (adalimumab) were applied. Despite the therapy, he died from heart failure and septicemia at 68th day of therapy.

Subcutaneous sarcoidosis with plantar involvement.

Sarcoidosis is a multisystem granulomatous disorder of an unknown etiology. Subcutaneous sarcoidosis is a rare manifestation of sarcoidosis, and plantar involvement is extremely rare and there is only one such case report in the medical literature. Herein we present an interesting case of a patient who was diagnosed as having subcutaneous sarcoidosis at a plantar localization because plantar involvement is extremely rare and also because of the successful outcome after performing intralesional corticosteroid therapy.

Ochronosis: complicated tear of black meniscus.

We describe a case of a previously healthy 35-year-old man who presents with meniscal symptoms, and present the arthroscopic findings of a complicated tear of black lateral meniscus. Investigations revealed that he had underlying alkaptonuria, which was previously undiagnosed without any other findings. After the surgical treatment, the patient's complaints were alleviated and almost no complaints were registered, during the next follow-up.

Primary cardiac angiosarcoma: a case report.

Primary tumors of the heart are rarely seen. Cardiac angiosarcomas are malignant tumors that almost always have a poor prognosis. We describe a 29-year-old man with primary cardiac angiosarcoma with multiple site metastases. The therapeutic approach includes surgery, chemotherapy and radiotherapy alone or in combination. New techniques of radiotherapy and combined chemotherapeutic agents may relieve symptoms and prolong a patient's life. We discuss the diagnosis and treatment of cardiac angiosarcoma in the light of a case report.

A case of radiation-induced sternal malignant fibrous histiocytoma treated with neoadjuvant chemotherapy and surgical resection.

BACKGROUND: Primary sternal malignant fibrous histiyocytoma (MFH) is highly rare. Effective treatment modality is surgical resection with wide margins. However, to date, the effects of radiotherapy or chemotherapy has not been clearly defined. CASE PRESENTATION: Herein, we aimed to present a 50-year old female patient with MFH occurred in the radiotherapy field who had had surgical procedure for breast cancer 19 years ago and had followed by radiotherapy. Neoadjuvant chemotherapy was applied for MFH due to cardiac and mediastinal vascular invasion. Wide resection was carried out for the mass after having been decreased in size following neoadjuvant chemotherapy. CONCLUSION: Neoadjuvant chemotherapy was an effective method. In planning the surgical resection, the size of the tumor before chemotherapy should be considered as the initial size and surgical margins should be determined accordingly.

Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients.

BACKGROUND: Secondary malignancies arising from benign bone tumors are rare. Their recognition and diagnosis are difficult, and their slow growth and late recurrence require long-term follow-up. In this study, malignant transformation rates of various histological types of benign cartilage-forming bone tumors in large series were evaluated. METHODS: Between 1986 and 2004, a retrospective analysis of 627 cartilage-forming benign bone tumors revealed that 32 patients had malignant transformation. Of the 32 patients, 14 had solitary osteochondromas, 10 had multiple osteochondromas, 6 had a solitary enchondroma, 1 had Ollier's disease, and 1 had Maffucci's syndrome. The patient with Ollier's disease had two chondrosarcomas, and one patient with multiple osteochondroma had three chondrosarcomas. The cases were included in the study only when complete clinical documentation, radiological records, and histological analyses were available. RESULTS: The rate of malignant transformation for cartilage-originating tumors was 5.1% (solitary osteochondromas 4.2%, multiple osteochondromas 9.2%, solitary enchondromas 4.2%). The average time between the initial diagnosis and malignant transformation was 9.8 years. The most common site of involvement was the proximal portion of the femur. The tumors generally were well differentiated. The mean follow-up period was 57.3 months. Five patients (15.6%) died of tumor recurrence or metastasis at an average of 20.6 months. One patient is alive with tumor at 104 months. CONCLUSIONS: Cartilage-forming benign bone tumors are rather prone to undergo malignant transformation. Although malignant transformation of a benign bone tumor is a rarely encountered situation, orthopedic surgeons should be cautious while following patients with a benign bone neoplasm. Early recognition and appropriate surgical treatment are required to achieve successful outcomes. The rate of local recurrence in secondary chondrosarcomas depends not only on adequate surgical treatment but also on the localization and histological grade.

Fine needle aspiration biopsy features with histologic correlation in mediastinal hepatoid yolk sac tumor presenting with sternum metastasis: a case report.

BACKGROUND: The hepatoid variant of yolk sac tumor (H-YST) is an exceedingly rare and highly malignant neoplasm. We present and discuss our experience with cytologic and histopathologic features of a mediastinal H-YST presenting with sternum metastasis, which to the best of our knowledge has not been previously reported. CASE: A 38-year-old man presented with a large mass on the sternum. Computed tomography of the thorax showed a large anterior mediastinal mass with sternum metastsis and multiple lung metastases. Laboratory examination revealed elevated serum alpha-fetoprotein (60,000 IU/mL). No tumor was found in the other organ systems. A percutaneous fine needle aspiration biopsy and subsequent open surgical biopsy were performed on the sternum metastasis. Cytologically, the tumor was composed of monotonous, large, round to polygonal hepatoid cells forming solid sheets and trabeculae entrapped with endothelial cells resembling hepatocellular carcinoma. Histopathologic sections of tumor showed tumor cells with eosinophilic to clear cytoplasm arranged in a solid, trabecular growth pattern, with some acinar formations. Immunohistochemical study supported the hepatoid origin. CONCLUSION: Fine needle aspiration cytology, together with the characteristic clinical presentations and specific tumor markers, is crucial to the initial diagnosis of H-YST.

Management of chondroblastoma: retrospective review of 28 patients.

BACKGROUND: This study retrospectively reviewed 28 patients who presented with chondroblastoma in our hospital during the period March 1986 to February 2006. We investigated factors associated with local recurrence of the tumors after surgery. METHODS: We recorded the patients' sex and age, the location of the pathology, imaging studies (status of growth plate and activity of lesions), histological findings, and treatment outcomes. RESULTS: Pain was the presenting symptom in all patients. The tumors were generally located in the epiphyses or apophyses of long bones, especially in the proximal humerus (9 patients), proximal tibia (7 patients), and proximal femur (5 patients). Our preferred treatment method was generally aggressive curettage of the lesion followed by bone grafting. There was local recurrence in six patients. A secondary aneurysmal bone cyst was present in two patients. CONCLUSION: Aggressive curettage and bone grafting resulted in local control in most patients. Tumor recurrence was not statistically associated with age, sex, growth plate status, tumor location, or tumor activity. The only parameter associated with increased local recurrence was previous surgery for chondroblastoma in the same location.

Ectopic thyroid gland on the ascending aorta with a partial pericardial defect: report of a case.

Intrathoracic ectopic goiters are rare. To our knowledge, only two reports of thyroid tissue on the aorta have been reported in the English literature. A 42-year-old woman was found to have a right paracardiac mass. Sternotomy revealed a firm and encapsulated tumor attached to the anterolateral surface of the ascending aorta and a defect in the right superior part of the pericardium. The mass was completely excised and histopathologic examination confirmed a multinodular goiter. We discuss the features of this rare tumor.

[Chondromyxoid fibroma: an evaluation of 11 patients]. / Kondromiksoid fibroma: 11 olgunun degerlendirilmesi.

OBJECTIVES: We evaluated surgical treatment of patients with chondromyxoid fibroma. METHODS: The study included 11 patients (6 females, 5 males; mean age 31 years; range 8 to 53 years) who underwent surgical treatment for chondromyxoid fibroma. The most common site of involvement was the tibia in three patients. Diagnosis was made preoperatively by tru-cut biopsies in seven patients and all the diagnoses were confirmed postoperatively by histopathologic examination. In addition to plain radiographs, computed tomography was used in 10 patients, and magnetic resonance was used in six patients. Surgery included wide resection, marginal excision or intralesional curettage followed by autologous bone graft or bone cement. The mean follow-up was 62.8 months (range 2 to 162 months). RESULTS: The main presenting symptom was pain in all the patients. Two patients with thoracic wall and tibia involvement, respectively, complained of a mass. Radiologic imaging showed soft tissue involvement in two patients. Recurrence occurred in three patients (27.3%), in whom initial surgical procedures were curettage alone (n=2) or with iliac graft (n=1). One patient with involvement in the phalanx of the thumb presented with pain 46 months after the second operation. A tru-cut biopsy yielded a diagnosis of secondary chondrosarcoma for which ray amputation was performed. No wound site infections or functional loss developed after surgical treatment. CONCLUSION: Chondromyxoid fibroma may develop in various bones of the body and occur at a wide age range. Curettage with autologous bone graft is an effective surgical method.

Management of fibrous dysplasia. A report on 36 cases.

A consensus on the clinical course of fibrous dysplasia has not yet emerged in the literature. We retrospectively evaluated 36 patients who were diagnosed with fibrous dysplasia in our institution and were followed for a mean duration of 56.5 months (range 7-210 months). Their mean age was 25.8 years (range 5-67 years); 46.7% were male. The most frequent presenting complaints were pain (66% of patients) and pathological fracture (20%). Osteosarcoma developed in one patient 20 years after he had undergone radiation therapy for fibrous dysplasia in the tibia. Mazabraud syndrome was encountered in two patients, and aneurysmal bone cyst associated with fibrous dysplasia was seen in one patient. Fibrous dysplasia is generally considered a static disease, but with long-term follow-up it is found to have a more dynamic nature. For this reason, patients with fibrous dysplasia should be followed carefully over the long-term.

Unusual extramedullary hematopoiesis in a patient receiving granulocyte colony-stimulating factor.

A 42-year-old woman was diagnosed with myelodysplastic syndrome with fibrosis that developed bilaterally, cervical lymphadenopathy and cutaneous infiltration by trilineage extramedullary hematopoiesis after granulocyte colony-stimulating factor therapy because of severe neutropenia. Hepatosplenomegaly was not observed during her follow-up. Extramedullary hematopoiesis disappeared after growth factor therapy was stopped. Although the neutropenia was alleviated by growth factor administration, the appearance of an unusual involvement of extramedullary hematopoiesis should be kept in mind.

Can specific vessel-based papulopustular lesions of Behçet's disease be differentiated from nonspecific follicular-based lesions clinically?

BACKGROUND: Although papulopustular lesions are one of the diagnostic criteria for Behçet's disease, controversy exists as to the nature of these lesions. Specific vessel-based papulopustular lesions as well as nonspecific follicular lesions may be seen in patients with Behçet's disease. Some authors suggest that papulopustular lesions should be considered a positive criterion only if they exhibit a vessel-based neutrophilic reaction. OBJECTIVE: [corrected] To determine whether specific vessel-based papulopustular lesions can be differentiated clinically from nonspecific follicular lesions in patients with Behçet's disease. METHODS: Twenty-three papulopustular lesions in 20 patients with Behçet's disease were initially examined clinically by two dermatologists blind to each other's diagnosis. Biopsies taken from these lesions were examined by a pathologist unaware of the patient data. RESULTS: Leukocytoclastic vasculitis or perivascular infiltration was observed in 10 lesions, perifollicular and perivascular infiltration was noted in nine lesions, and perifollicular inflammation was seen in four biopsy specimens. Most of the lesions interpreted clinically as specific papulopustular lesions of Behçet's disease had predominantly perivascular infiltration or leukocytoclastic vasculitis; however, three papulopustular lesions evaluated by both observers as specific papulopustular lesions had only perifollicular inflammation, and one lesion diagnosed clinically as a nonspecific follicular eruption revealed perivascular neutrophilic reaction. Interobserver variance was noted in three papulopustular lesions. CONCLUSIONS: Clinical examination may not be sufficient to predict the dermatopathologic pattern in all lesions. Papulopustular lesions with no specific clinical and histopathologic features may create problems in the diagnosis of Behçet's disease.

Cytopathologic features of pituitary carcinoma with cervical vertebral bone metastasis: a case report.

BACKGROUND: Pituitary carcinomas are extremely rare tumors of the adenohypophysis. The presence of craniospinal and/or systemic extracranial metastases is the only reliable criterion for the diagnosis of pituitary carcinoma. To date, only 2 cases have been reported correctly by fine needle aspiration biopsy (FNAB). We present an additional case of pituitary carcinoma with FNAB features. CASE: A 60-year-old woman presented with clinical features of Cushing's disease and a pituitary tumor. She underwent transsphenoidal resection of the tumor. The initial diagnosis was an adrenocorticotrophic hormone (ACTH)-producing invasive pituitary adenoma. The patient presented again with neck pain 6 years after the operation. Magnetic resonance imaging revealed metastatic tumor masses at the level of C5-C6 of the cervical vertebrae. Intraoperative fine needle aspiration and incomplete excision of metastatic tumors were performed. Cytologically, tumor cells were composed of a combination of loose groups and single cells. Neoplastic cells had a relatively monotonous appearance and displayed characteristic neuroendocrine tumor features. Immunocytochemistry from cell block sections revealed AE1/ AE3, synaptophysin chromogranin A and ACTH positivity in the tumor cells. CONCLUSION: Pituitary carcinoma with extracranial systemic metastases demonstrates typical neuroendocrine features on fine needle aspiration. In the differential diagnosis, metastatic neuroendocrine carcinomas should be kept in mind. In the absence of sufficient clinical data, these 2 entities cannot be distinguished correctly through the cytologic features.

Abdominal Castleman disease with mixed histopathology in a patient with iron deficiency anemia, growth retardation and peliosis hepatis.

In Castleman disease, also known as giant lymph node hyperplasia, lesions are usually located in the chest. Peliosis hepatis is the presence of multiple blood-filled cysts in the liver. The combination of these two disorders in one patient is rare, with only three cases reported to date. We report the case of a 21-year-old man who presented with growth retardation and iron deficiency anemia. Work-up revealed hepatomegaly and multiple conglomerated giant lymph nodes near the splenic hilum. Once the mass was surgically removed, the patient's anemia rapidly resolved and he showed catch-up growth. The lymphoid tissue exhibited mixed histologic type, so the patient was diagnosed with both hyaline vascular type and plasma cell type Castleman disease. A liver biopsy obtained at surgery showed sinusoidal dilatation and blood-filled cysts, indicating peliosis hepatis. This is the fourth documented case of peliosis hepatis associated with Castleman disease. In addition to describing the case features, we discuss the possible pathogenesis of peliosis hepatis in the setting of Castleman disease.