Incidence of osteosarcoma, chondrosarcoma and Ewing's sarcoma in east Denmark: Reverse male to female ratio in osteosarcoma

Summary The incidence of osteosarcoma, chondrosarcoma and Ewing's sarcoma was studied in east-Denmark during a 14 years period from 1983 to 1996. The population of east Denmark was 2.3 millions people. A total of 109 primary osteosarcoma, chondrosarcoma and Ewing's sarcomas were found. The mean age of the 47 patients (15 women and 32 men) with chondrosarcoma was 54 years (range 30–85 years). The mean age of the 45 patients (23 women and 22 men) with osteosarcoma was 26 years (range 6–72 years). The mean age of the 17 patients (7 women and 10 men) with Ewing's sarcoma was 19 years (range 3–33 years). The overall incidence of chondrosarcoma was 1,46/1000000 inhabitants/year. The overall incidence of osteosarcoma was 1,40/1000000 inhabitants/year. The overall incidence of Ewing's sarcoma was 0,53/1000000 inhabitants/year.     

Soft Tissue Tumors Rarely Presenting Primary in Bone; Diagnostic Pitfalls

Primary bone sarcomas represent extremely rare entities. The use of now abolished labels, such as malignant fibrous histiocytoma and hemangiopericytoma, has significantly hampered the chance of identifying specific entities. It is now accepted that a broad variety of mesenchymal malignancies most often arising on the soft tissue may actually present as primary bone lesions. A more accurate morphologic partition is justified based on availability of distinct therapeutic options. An integrated diagnostic approach represents the only way to achieve a correct classification. In consideration of the significant complexity, primary bone sarcomas should ideally be handled in the context of expert centers.     

Head and Neck Soft Tissue Sarcomas: A Multivariate Analysis of Outcomes

Background Soft tissue sarcomas of the head and neck region represent a rare group of tumors of which a limited number of published individual- and institution-based experiences exist.Methods We performed an analysis of head and neck sarcoma patients identified from our institution between 1973 and 1999. Exclusion criteria included pediatric rhabdomyosarcomas, sarcomas of the neuromeningeal axis or non–head and neck primary disease sites, and bone sarcomas. All cases underwent pathologic re-review before statistical analysis.Results After pathologic review, 111 head and neck sarcoma patients remained (mean age, 47 ± 20 years). The median duration of follow-up was 51 months; the actuarial 5-year relapse-free, disease-specific, and overall survivals were 55%, 52%, and 44%, respectively. Forty-six percent remained free of recurrence at the most recent follow-up, and the most common site of recurrence was local followed by distant sites. By multivariate analysis, size and grade significantly influenced relapse-free, disease-specific, and overall survivals, whereas margin status additionally influenced relapse-free survival. Subset analysis of the fibrosarcoma/malignant fibrous histiocytoma and desmoid/dermatofibrosarcoma protuberans histologies was undertaken.Conclusions Size >5 cm and high-grade histology are considered poor prognostic indicators. Patients with either of these characteristics should be considered for adjuvant trials.     

Neuroendocrine Neoplasms of the Head and Neck: Some Suggestions for the New WHO Classification of Head and Neck Tumors

As knowledge and understanding in pathology evolve, classifications and nomenclature also change to reflect those advances. The 2005 World Health Organization Classification of Head and Neck Tumours was a significant step towards diagnostic standardization of head and neck neuroendocrine carcinomas; however, in the last 10 years there have been new data supporting the recognition of “large cell neuroendocrine carcinoma” as a distinctive high grade carcinoma in the head and neck, a lesion not included in the 2005 Classification. In addition, the terms “middle ear adenoma” and “carcinoid tumor of middle ear” are still widely used to describe a neoplasm that is neither a pure adenoma nor a carcinoid tumor but a lesion with variable mixed exocrine and endocrine differentiation. Largely using the diagnostic criteria of the WHO classification of neuroendocrine carcinomas of the lung, we propose the terms “neuroendocrine carcinoma, grade 1”; “neuroendocrine carcinoma, grade 2”; “neuroendocrine carcinoma, grade 3, large cell type”; and “neuroendocrine carcinoma, grade 3, small cell type” for the classification of neuroendocrine carcinomas of the head and neck in a future WHO classification. In addition, we also proposed the term “mixed epithelial neuroendocrine tumor” of the middle ear as an alternative for “middle ear adenoma” and “carcinoid tumor of the middle ear”.     

Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

Lymphoid and histiocytic lesions of the head and neck in pediatric patients is a fascinating topic as most of these lesions are benign, but that the neoplastic cases are essential to diagnose accurately for appropriate treatment. It is thought that 90% of children will have palpable lymph nodes between the ages of 4 to 8; most, but not all, are non-malignant and some resolve spontaneously without treatment. This paper will look at many of the benign and malignant lesions of both lymphocytic and histiocytic origin that present in the head and neck of children focusing on their diagnostic criteria. There is a very pertinent discussion of nonmalignant lymphoid proliferations, as infections and other reactive conditions dominate the pathology of pediatric lymphohistiocytic head and neck lesions. Discussion of those lymphomas which arise more frequently in the head and neck focuses on those seen in children and young adults such as classic Hodgkin lymphoma and Burkitt lymphoma, as well as new more controversial entities such as pediatric-type follicular lymphoma. Histiocytic lesions, both benign and malignant, are described and may be challenging to diagnose.     

Osteoprotegerin and Bone Mass in Squamous Cell Head and Neck Cancer Patients

Osteoprotegerin (OPG) is considered one of the main regulators of bone remodeling. Various patterns of serum OPG levels have been described in different types of tumors. We undertook this study to determine serum OPG levels in patients with squamous cell head and neck cancer (SCHNC), analyzing their relationship with other metabolic bone parameters and bone mineral density (BMD), as well as the possible influence of chemotherapy. Forty male patients with localized SCHNC were studied, and their results were compared with those of 40 healthy male controls. The type of treatment followed by each patient was noted. Age, weight, height, and lifestyle habits were recorded; and OPG, Ca²⁺, intact parathyroid hormone (iPTH), 25-Hydroxyvitamin D (25OHD) and 1,25-Dihydroxyvitamin D (1,25(OH)₂D), bone alkaline phosphatase, osteocalcin, and serum C-terminal cross-links telopeptide of type I collagen (ICTP) were determined. Dual-energy X-ray absorptiometry BMD at the lumbar spine, femoral neck, and hip was also measured. Serum OPG was higher in patients than in controls (91.7 ± 25.8 vs. 77.2 ± 26.3, P = 0.02). ICTP (a bone resorption marker) was 37% higher in patients (P = 0.007). Bone mass was lower in patients at the lumbar spine, femoral neck, and total hip. Lumbar spine Z-score showed a significant progressive decrease in controls, stage I-III patients, and stage IV patients. Logistic regression analysis showed a significant association between the disease and serum OPG levels, the odds ratio per standard deviation increase of this being 1.9 (95% confidence interval 1.1–3.8, P = 0.04) after adjusting for bone mass and ICTP serum levels, as well as for alcohol and smoking history. Adjustment for alcohol intake and tobacco use did not cancel out BMD differences between patients and controls. Patients with SCHNC show increased OPG serum levels, increased bone resorption, and decreased bone mass. The OPG rise appears to be unrelated to the BMD decrease, and the BMD decrease seems to be, at least in part, independent of smoking and drinking habits. No differences in either OPG or BMD were seen between patients with and without chemotherapy. Further studies are needed to clarify the mechanisms responsible for OPG and BMD changes in SCHNC.     

Cutaneous Head and Neck Squamous Cell Carcinoma with Regional Metastases: The Prognostic Importance of Soft Tissue Metastases and Extranodal Spread

Background  

Extranodal spread (ENS) is an established adverse prognostic factor in metastatic cutaneous squamous cell carcinoma (cSCC); however, the clinical significance of soft tissue metastases (STM) is unknown. The aim of this study was to evaluate the prognosis of patients with STM from head and neck cSCC, and to compare this with that of node metastases with and without ENS.     

Review of Pediatric Head and Neck Neoplasms that Raise the Possibility of a Cancer Predisposition Syndrome

Cancer predisposition syndromes (CPS) are generally heritable conditions that predispose individuals to develop cancer at a higher rate and younger age than their representative general population. They are a significant cause of cancer related morbidity and mortality in the pediatric population. Therefore, recognition of lesions that may be associated with a CPS and alerting the clinicians to its implications is a crucial task for a diagnostic pathologist. In this review we discuss benign pediatric head and neck lesions associated with CPS namely: odontogenic keratocyst, juvenile nasopharyngeal angiofibroma, ossifying fibroma of the jaw, paraganglioma, plexiform neurofibroma, plexiform schwannoma, mucosal neuroma, and nevus sebaceous syndrome; along with malignant tumors such as squamous cell carcinoma. Several head and neck melanocytic, endocrine, and central nervous system tumors can also be associated with CPS; they are beyond the scope of this article. Nasal chondromesenchymal hamartoma is discussed elsewhere in this issue.     

Infectious Pseudotumors: Red Herrings in Head and Neck Pathology

Infectious pseudotumors are tumor-like growths caused by specific microbial organisms. Proliferating histiocytes in these lesions may show atypical cytology with nuclear pleomorphism and increased mitotic activity, erroneously suggestive of malignancies including carcinoma, sarcoma, and lymphoma. Specific and nonspecific immunohistochemical staining profiles may lead to the consideration of a wide range of benign and malignant neoplastic processes. Two such cases are reported. The first is an obstructive endotracheal mass in an AIDS patient caused by Rhodococcus equi infection. The proliferating histiocyes were cytologically atypical with deeply eosinophlic granular cytoplasm leading to the consideration of oncocytic carcinoma, Hurthle cell carcinoma, and pleomorphic rhabdomyosarcoma. The second case is a nasal mass with a microscopic and immunohistochemical profile suggestive of chordoma and paraganglioma. Special microbial stains revealed intracellular bacilli consistent with Klebsiella rhinoscleromatis. In both cases, microbial virulence factors affecting phagocytosis prolonged their intracellular survival and resulted in active histiocytic proliferation. It is of importance that the surgical pathologist be conscious that some infectious processes can clinically and microscopically mimic malignant neoplasms. Accurately identifying these lesions and the specific causative agent is of particular significance since they can be successfully treated with antibiotics.     

骨段滑移术治疗胫骨缺损合并软组织缺损

目的总结骨段滑移术治疗胫骨长节段骨缺损合并小腿软组织缺损的疗效、适应证及术后康复在促进功能恢复中的作用。方法在2005年1月到2011年1月6年间治疗胫骨长节段性骨缺损合并小腿软组织缺损共13例,男性10例,女性3例;年龄16～35岁,平均24岁。胫骨缺损部位在胫骨中下段11例,在胫骨中上段2例。缺损长度7～15cm,平均9cm。软组织缺损位于小腿的前内侧,范围3cm×2cm～18cm×10cm。采用Orthofix重建外固定架,一期截骨,7d后开始延长,每日1mm,到胫骨远近缺损骨端紧密对合,维持固定直至骨愈合。小腿软组织缺损感染重、渗出多者使用负压封闭引流技术。术后进行康复治疗。结果从开始治疗到去除外固定架,治疗用时11～23个月,平均18个月。13例胫骨骨缺损获得重建,患肢肢体长度与健侧相差小于2cm,截骨延长新生骨部分愈合良好。11例骨缺损接触端自行愈合,有2例骨折断端软组织内陷阻止骨端接触,1例采用软组织松解,1例行软组织松解加自体植骨术。创面均得到覆盖。闭合的创面部分凹陷为贴骨瘢痕,遇阴雨天不适。骨段滑移过程中在牵开3cm左右时患者感到小腿疼痛,对症治疗后大多可继续进行延长,有4例停止延长3～5d后继续延长直至完成。外固定架未出现固定钉明显松动现象,中间2枚固定钉在滑移的中后期有不同程度的对皮肤切割现象,将皮肤钉孔拉成椭圆形,此钉孔在骨段滑移停止后3周左右恢复正常。所有患者膝关节活动正常,踝关节背伸活动可达15°～30°。结论骨段滑移术是治疗胫骨长节段骨缺损合并软组织缺损的一种较好的方法,最适合的病例是胫骨中上段或中下段长节段骨缺损合并软组织缺损,胫骨近端和远端有置入固定钉的足够长度,腓骨完整性较好的患者。结合康复治疗可使伤残肢体功能最大限度地恢复。