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Carney complex (CNC) is a multiple neoplasia syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine and non-endocrine tumors. Most of the cases have an inactivating mutation in the PRKAR1A gene. Osteochondromyxoma (OMX) is an extremely rare myxomatous tumor of bone, affecting 1% of CNC patients. Large cell calcifying Sertoli cell tumor (LCCSCT) is a testicular tumor affecting more than 75% of males with CNC. Here, we report an atypical case of CNC without typical pigmented skin lesions, presenting with a bone based tumor as the first manifestation. Initial presentation was for a recurrent, locally invasive intranasal tumor without definite diagnosis. Further clinical developments during follow up, central precocious puberty and testicular tumor with calcification, led to the diagnosis of LCCSCT, a CNC-related tumor. Histopathologic examination of the intranasal tumor was re-evaluated with this knowledge and OMX was diagnosed. Coexistence of OMX and LCCSCT suggested CNC. Genetic analysis revealed a heterozygous non-sense p.Trp 224* (c.672G>A) in the PRKAR1A gene. In our case, the diagnosis of OMX was delayed, because it is extremely rare and little is known about this tumor. Thus the aim of this report was to alert other clinicians to consider CNC if OMX is diagnosed.

Objective: The aim of this study was to define histopathological features of giant cell tumor of bone, especially accompanying fibrohistiocytic or aneurysmal bone cyst like components, in the light of our institutions experience., Methods: A total of 120 cases (64 females and 56 males; mean age: 36.2 (12-80)) with 'GCT' diagnosed between the years 1996-2016 were included in this retrospective analysis. Cases were evaluated according to clinical features such as age, gender, localization, recurrence, metastasis and histopathological features., Results: Tumors were localized most frequently at proximal tibia and distal femur, respectively. In 11 cases areas rich in fibrohistiocytic component and in 20 cases aneurysmal bone cyst like component were observed. In 2 cases both components were present. Twenty three cases recurred. In 1 case which was primarily located at calcaneus, tumor metastasized to lung 4 years later during follow-up., Conclusion: GCT can be confused with other tumor or tumor-like lesions involving giant cells. Secondary changes such as fibrohistiocytic or aneurysmal bone cyst-like components and coagulation necrosis were frequently seen in conventional giant cell tumor of bone. For tumors having prominent fibrohistiocytic and/or aneurysmal bone cyst-like components, in order to detect characteristic areas representing GCT, additional sampling is essential. Although secondary histopathological changes do not appear to affect clinical outcome, these features are important in differential diagnosis. Approximately one fifth of GCT cases show recurrence and sacrum and foot bones were the most frequent sites for recurrence., Level of Evidence: Level IV, diagnostic study., (Copyright © 2018 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.)

Osteosarcoma (OS) is a malignant bone tumor and it is very rare in head and neck region. If it arises in this area, most common localizations of this tumor are mandible and maxilla and ethmoid sinus is very rare site for this malignancy. A 43-year-old female admitted to our clinic with the complaint of pain in the right half of the face and headache persisting for 2 months. According to the clinical symptoms and imaging studies, sinonasal ossifying fibroma was suspected. For this reason, patient was operated endoscopically and histopathological examination revealed that osteoid matrix producing atypical osteoblastic cells so these findings identified osteoblastic variant OS. This article is about a very rare case of OS of ethmoid sinus. The differential diagnosis, general characteristics, and classification of this tumor, radiological features, and surgical techniques are also presented.