Squamous carcinoma arising in a branchial cleft cyst: have you ever treated one? Will you?

The existence of primary branchiogenic carcinoma - that is, carcinoma arising in a pre-existing branchial cleft cyst (a benign developmental cyst) - has in recent decades been the subject of increasing scepticism. Recognition of the propensity of a variety of head and neck sites - including in particular the tonsil - to give rise to cervical metastases while the primary tumours themselves remain undetected has given rise to the idea that virtually all cystic carcinomas of the neck represent metastatic deposits, whether or not their primary sites are found. A diagnosis of primary branchiogenic carcinoma should be viewed with extreme scepticism, and every effort should be made (e.g. imaging, panendoscopy, elective tonsillectomy) to exclude the existence of a primary site elsewhere, before considering a diagnosis of primary branchiogenic carcinoma.

Chemotherapy- and radiotherapy-induced intestinal damage is regulated by intestinal trefoil factor.

BACKGROUND & AIMS: Injury to the intestinal mucosa is frequently a dose-limiting complication of radiotherapy and chemotherapy. Approaches to limit the damage to the intestine during radiation and chemotherapy have been largely ineffective. Trefoil factors are produced throughout the gastrointestinal tract and regulate cell migration, restitution, and repair. Studies were undertaken to define the role of intestinal trefoil factor in modulating the intestinal response to chemotherapy and radiation. METHODS: The effect of intestinal trefoil factor on migration and cell survival in intestinal epithelial monolayer exposed to methotrexate was studied in vitro. Chemotherapy and radiation damage was assessed in wild-type and intestinal trefoil factor-null mice in the presence or absence of supplemental intestinal trefoil factor administered in drinking water. RESULTS: Radiation and chemotherapy induced a marked reduction in goblet cell number and intestinal trefoil factor messenger RNA, as well as intestinal trefoil factor promoter activity. Intestinal trefoil factor improved intestinal epithelial cell viability and wound repair after chemotherapy exposure in vitro. Intestinal trefoil factor-deficient mice (intestinal trefoil factor(-/-)) were more susceptible to chemotherapy- and radiation-induced mucositis. Oral recombinant intestinal trefoil factor reduced the severity of both chemotherapy-induced and chemotherapy/radiotherapy-induced intestinal mucositis. CONCLUSIONS: These studies suggest that intestinal trefoil factor is involved in protection against and recovery from intestinal mucositis induced by radiation and chemotherapy.

Pathologic detection of occult metastases in regional lymph nodes in patients with head and neck cancer.

Squamous carcinomas of the head and neck region are exceedingly common problems in the routine practice of head and neck tumour surgery. Well-defined treatment protocols have evolved to manage patients afflicted with such tumours. This article explores the role of the hospital pathologist in the detection of occult metastases. The conventional approach to evaluation of cervical nodes from a neck dissection is reviewed, and then the potential utility of more recently developed diagnostic approaches (such as immunohistochemistry and polymerase chain reaction) is explored.

The pathology of neck dissection in cancer of the larynx.

Cancer of the larynx is a common problem in a head and neck oncological surgical practice; as such, pathology departments supporting such surgical practices will examine cervical lymph node dissection specimens with some frequency. Issues to be settled among pathologists and surgeons include--How precise an anatomic dissection of the specimen is called for? What histological features of the specimen will be of most use to the clinicians who are devising a course of postoperative therapy for the patient? What sorts of methods are needed to identify the maximum number of micrometastases which may be lurking within the lymph nodes of the specimen? Is there a role for routine application of special techniques--such as immunohistochemistry or molecular biology--in the analysis of these specimens? While the answers to these questions are likely to vary somewhat from one center to another, patients are best served when these questions are discussed amongst the respective physicians before surgical procedures are undertaken, rather than after the fact.

Management of the clinically negative cervical lymph nodes in patients with non-conventional squamous carcinoma of the larynx.

This paper discusses the indications for neck dissection in those patients with laryngeal tumours which prove to be, on pathologic examination, not conventional squamous carcinomas but rather fall into the category either of one of the uncommon histological subtypes of carcinoma, or another type of unusual histological variant of laryngeal malignancy, a mesenchymal tumour. It appears as though these unusual laryngeal malignancies may manifest both biological behaviours and propensities to metastasize to regional lymph nodes that differ from those of the more commonly encountered conventional squamous carcinomas of the larynx. This heterogeneous group of tumours accounts for about 10 per cent of all malignant tumours of the larynx.

Review of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of head and neck.

The entity known as sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RD disease), is an uncommon benign proliferation of hematopoietic and fibrous tissue that often presents in the head and neck region. Its initial manifestations most often include a roughly symmetric, painless, bilateral cervical adenopathy, although extranodal disease may develop in a minority of patients. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes ("emperipolesis"); these distinctive large, pale cells - RD cells - are S-100 protein-positive by immunostaining and so differ from ordinary histiocytes. Despite its sometimes impressive clinical presentation, SHML is a benign and self-limited disease, whose treatment is aimed largely at controlling its local manifestations (most often by surgical therapy). The microscopic differential diagnosis, particularly in extranodal disease, is at times challenging and can include Langerhans' cell histiocytosis, Hodgkin's disease, non-Hodgkin's lymphoma, metastatic carcinoma, and metastatic malignant melanoma.

Lipomatous hemangiopericytoma: a rare variant of hemangiopericytoma that may be confused with liposarcoma.

We describe the clinicopathologic features and biologic behavior of 16 cases of histologically benign hemangiopericytoma containing a variable amount of mature fat as an intrinsic part of the neoplasm. These so-called lipomatous hemangiopericytomas occurred primarily in men (12 men and 4 women) with a mean age of 54 years (range, 33-74 years). All occurred in deep soft tissue and had an average size of 10 cm when first detected. All were characterized by a relatively sharp border and typical histologic features of hemangiopericytomas, including oval to round cells surrounding a sinusoidal and staghorn vasculature often with perivascular hyalinization. Mature fat varied in amount but usually occupied approximately one quarter to three quarters of the area of tumor. Mitotic activity was low, with more than half the cases having no mitotic activity. Five cases showed moderate nuclear atypia. In four cases, the pericytic regions had sclerotic zones. In contrast to liposarcoma, neither lipoblasts nor isolated atypical hyperchromatic cells within mature fat, as are seen in well-differentiated liposarcoma, were present. Immunohistochemistry performed in four cases showed factor XIIIa in tumor cells and an intricate pattern of immunoreactivity around cells for type IV collagen. CD34 and smooth-muscle actins were identified in two of four cases. Follow-up in seven cases showed no recurrences or metastases within the follow-up period of 1 to 7 years. Because these lesions are located in deep soft tissue and contain large amounts of mature fat, they could be mistaken for well-differentiated liposarcomas in limited biopsy material, although the distinction is easily made in examining the entire specimen. The lipomatous hemangiopericytoma represents yet another example of a bimodal mesenchymal tumor containing mature fat and raises the question of whether a common cytogenetic abnormality can explain the emergence of two clonal populations in this hybrid tumor.

A review of heterotopia and associated salivary gland neoplasms of the head and neck.

Salivary tissue neoplasms may involve normal, accessory and heterotopic salivary gland tissue. A case of Warthin's tumour originating from heterotopic salivary gland tissue of the upper neck is reported. The radioactive uptake of 131I, evidenced in the neck mass in its pre-diagnostic assessment, suggested a diagnosis of cervical node involvement from a primary malignant thyroid neoplasm. A critical review of the literature on heterotopic salivary gland tissue neoplasms of the head and neck is also presented.

Subglottic carcinoma: review of a series and characterization of its patterns of spread.

The rarity of primary subglottic malignancies, along with the varied definitions of the anatomic confines of this region, have limited our understanding of the patterns of tumor spread within the subglottis. We conducted a retrospective chart review to analyze clinical and pathologic data in patients with subglottic carcinoma. A pattern of disease progression was identified, which is defined by the cartilaginous laryngeal framework, with the fibroelastic barriers susceptible to tumor invasion. We conclude that although cartilaginous laryngeal structures are preserved until late in the disease course, the ability of tumors to invade the fibroelastic membranes provides them with an insidious means of escape. Specifically, tumor progression occurs primarily within the paraglottic space and extralaryngeal compartments; the potential for mucosal spread is limited. The lack of mucosal disease in patients whose cartilaginous laryngeal structures are intact may present a facade of normality in patients with advanced disease, and perhaps delay the early diagnosis of subglottic malignancies by physical and radiologic examination.

The trefoil gene family are coordinately expressed immediate-early genes: EGF receptor- and MAP kinase-dependent interregulation.

The trefoil gene family of mucus cell-secreted proteins is a critical mediator of gastrointestinal mucosal restitution. Transcription of trefoil genes is induced during mucosal repair, but the regulatory mechanisms involved are unknown. Mice deficient in the intestine-specific peptide intestinal trefoil factor (ITF), in which colonic restitution is lethally impaired, showed reduced expression of the gastric trefoil genes SP and pS2, suggesting that trefoil peptides may individually regulate transcription of the entire family. In gastric cell lines, the trefoils were shown to act in a manner suggestive of immediate-early genes capable of auto- and cross-induction through cis-acting regulatory regions. Trefoil-mediated transcriptional regulation required activation of the Ras/MEK/MAP kinase signal transduction pathway. EGF receptor (EGF-R) activation was also necessary for trefoil auto- and cross-induction, and both spasmolytic polypeptide (SP) and ITF stimulation of gastric cell lines led to phosphorylation of EGF-R. Nevertheless, ITF and ITF-thioredoxin cell surface binding at 4 degrees C colocalized not with EGF-R, but with CD71, which is found in clathrin-coated pits, suggesting that integration of trefoil peptide responses may occur after internalization. As EGF-R expression is itself strongly induced after mucosal damage, the trefoil/EGF-R relationship may be pivotal in the generation and maintenance of the mucosal repair phenotype.

Sinonasal malignant lymphomas: a distinct clinicopathological category.

Sinonasal lymphomas represent a distinct subset of extranodal head and neck lymphomas. While sinonasal lymphomas are relatively rare in Western countries, in Asian populations they are the second most frequent group of extranodal lymphomas, after gastrointestinal lymphomas. With advances in immunohistochemistry, these lymphomas have been separated into B-cell, T-cell, and most recently into natural killer (NK) cell phenotypes. The B-cell phenotype is typically located in the paranasal sinuses and has a slight predominance in Western countries. The T/NK-cell phenotype is the most common in Asian and South American countries. These tumors are typically located in the nasal cavity and have an aggressive, angioinvasive growth pattern that often results in necrosis and bony erosion. Thus, sinonasal lymphomas have been included in the past with other destructive malignant and benign lesions under the descriptive and nonspecific name lethal midline granuloma. Patients are classically in the sixth to eighth decades, with a 2:1 male-to-female ratio. The prognosis is generally better than that of nodal-based lymphomas of similar histologic grade. Treatment is a combination of local irradiation and chemotherapy with an anthracycline-based regimen.

Sarcoidosis presenting as a tumorlike muscular lesion. Case report and review of the literature.

Sarcoid myopathy presenting as a tumorlike lesion is an exceedingly rare presentation of sarcoidosis. Concurrent extramuscular involvement is common. Chest radiographs, if abnormal, may suggest the diagnosis. Magnetic resonance imaging is the preferred study for diagnosis and follow-up of tumorous sarcoid myopathy. Optimal therapy is not clear. Favorable responses have been cited with surgery or corticosteroids (alone or in combination). Azathioprine or alternative immunosuppressive agents (for example, antimalarials or methotrexate) may have a role in corticosteroid-recalcitrant patients. The role of local radiotherapy is controversial and should be reserved for severe localized disease refractory to aggressive medical therapy.

Papillary squamous cell carcinoma versus verrucous squamous cell carcinoma of the head and neck.

Papillary squamous cell carcinoma and verrucous squamous cell carcinoma of the head and neck may be confused. The clinicopathological profile of the two neoplasms is presented and the differential diagnosis is discussed. A correct diagnosis is imperative in order to institute the most appropriate treatment.

Squamous cell carcinoma of the buccal mucosa.

The purpose of this study was to establish treatment criteria for patients with early-stage squamous cell carcinoma of the buccal mucosa. Thirty-one patients were analyzed in a retrospective fashion. Distribution of patients according to tumor stage was relatively even. Within 5 years recurrent disease developed in nearly 80% of evaluable patients. There was a 100% overall incidence of local disease recurrence for patients with stage I and II tumors treated with wide local excision alone and followed up for more than 2 years. On the basis of these data, we conclude that wide local excision for early-stage buccal carcinoma is associated with a high local failure rate. Possible causes for failure and alternative treatment approaches are discussed.

Pediatric myofibromatosis of the head and neck.

OBJECTIVES: To examine the clinical and pathological features of pediatric myofibroma of the head and neck and to discuss the challenges in diagnosis and treatment. DESIGN: A retrospective search of pathology department and clinical records to identify patients with myofibroma and a retrospective review of English-language medical publications. SETTING: Academic medical center. PATIENTS: Thirteen pediatric patients (aged from birth to 8 years old) diagnosed as having myofibroma of the head and neck. RESULTS: Nine of 13 patients were cured with conservative surgical excision. Four patients (31%) had recurrence, requiring multiple surgical procedures. One third showed spontaneous regression clinically or by histological examination. The clinical course did not parallel the histological appearance, as high cellularity and mitotic figures were commonplace among the specimens. A misdiagnosis of malignancy was not unusual in this series, as 3 patients had an initial diagnosis of fibrosarcoma, which on review was revised to myofibroma. CONCLUSIONS: Myofibromatosis is a distinct disorder among the great number of fibrous proliferations occurring in infants and children, with a particular predilection for the head and neck region. These lesions should be clearly distinguished from conventional adult-type fibromatoses (desmoid tumors), which are more aggressive. Most patients have solitary lesions that respond well to conservative surgical excision, whereas a few of these lesions behave more aggressively, requiring several surgical procedures for the management of recurrent or persistent tumor. Many of these lesions show spontaneous regression, suggesting that lesions not affecting vital functions, resulting in growth anomalies, or demonstrating rapid aggressive growth may be managed conservatively.