Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.     

A Case of Cutaneous Malakoplakia in the Head and Neck Region and Review of the Literature

Malakoplakia is a rare inflammatory condition that typically occurs in the urinary tract. The cutaneous form is less prevalent, and most commonly occurs in the perianal or genital regions. Here we present a 61 year old lady with cutaneous malakoplakia of the neck, which was successfully treated with surgical excision and a prolonged course of ciprofloxacin. We follow our case report with a discussion and literature review of all seventeen previously reported cutaneous head and neck malakoplakia cases from the literature. A diagnosis of cutaneous malakoplakia should be considered in nodular, ulcerated or discharging lesions that are refractory to treatment. Histology is essential, not only to diagnose malakoplakia, but also to exclude other important differential diagnoses, such as malignancy. Combined surgical excision and prolonged antibiotic courses appear to have the highest success rate. Antibiotics should be culture specific, but quinolones appear to be the best empirical choice.     

Head and Neck Extranodal Interdigitating Dendritic Cell Sarcoma: Case Report and Review of the Literature

Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm originating from professional antigen presenting cells normally located in the T zone of the lymph node. The purpose of this report was to describe the first case of the IDCS of the submandibular gland and perform a review of the literature of head and neck IDCS. We present a case of an 81-year-old man with a 5 months history of slowly enlarging painless mass in right submandibular region. Fine needle aspiration cytology was suggestive of squamous cell carcinoma. The patient underwent surgical resection of the right submandibular gland and neck dissection. A malignant spindle cell proliferation involving the submandibular gland and colonizing one laterocervical lymph node was found. Morphology and immunophenotype prompted a differential diagnosis of a metastatic spindle cell melanoma versus an IDCS. Transmission electron microscopy was performed and supported a diagnosis of IDCS. The diagnosis of IDCS is a challenging task and may require a large array of techniques.     

Talar Neck Fractures: A Systematic Review of the Literature

Considerable controversy surrounds the management of talar neck fractures regarding the rate of post-traumatic arthrosis, secondary procedures, avascular necrosis, and the effect of the interval to surgery on these variables. A data search using PubMed was performed with the keywords “talus” and “fracture.” The search found 1280 studies. Ultimately, 21 reports involving 943 talar neck fractures were analyzed. Data concerning open fractures, the interval to surgery and its relationship to the incidence of avascular necrosis, and the rates of malunion and nonunion, post-traumatic arthrosis, secondary salvage procedures, and functional outcomes were collected and analyzed. The variables examined were not uniformly reported in all studies. The overall rate of avascular necrosis was 33%, with no demonstrated relationship between the interval to surgery and the rate of avascular necrosis. Malunion occurred approximately 17% of the time, with nonunion occurring approximately 5% of the time. Post-traumatic arthrosis occurred in 68% of patients, although secondary salvage procedures were only performed in 19% of patients. Functional outcomes were difficult to assess, given the variability of reported outcomes and unvalidated measures. The optimal management of talar neck fractures has yet to be determined. Although the present review has improved understanding of these difficult fractures, additional studies that use validated outcomes measures are warranted to determine the effect of delayed surgery on final outcomes and optimal treatment methods.     

Head and Neck Reconstruction in Patients with Polycythemia Vera: Case Series and Literature Review

Background  Polycythemia vera (PV) is a myeloproliferative disease with overproduction of erythrocytes, leukocytes, and platelets causing an increased risk of both thrombosis and hemorrhage. There are limited reports and no established guidelines for managing such patients undergoing reconstructive surgery. Methods  We present four patients with PV and head and neck cancer who required reconstruction after resection and provide a review of the current literature. Results  Preoperatively, patients on cytoreductive therapy continued with their treatment throughout their hospital course and had hematologic parameters normalized with phlebotomy or transfusions if needed. Two patients who underwent free flap surgery (cases 1 and 2) had postoperative courses complicated by hematoma formation and persistent anemia, requiring multiple transfusions. Cases 3 and 4 (JAK2+ PV and JAK2− PV, respectively) underwent locoregional flap without postoperative complications. Conclusion  Concomitant presentation of PV and head and neck cancer is uncommon and presents unique challenges for the reconstructive surgeon. Overall, we recommend that patients should have hematologic parameters optimized prior to surgery, continue ruxolitinib or hydroxyurea, and hold antiplatelet/anticoagulation per established department protocols. It is essential to engage a multidisciplinary team involving hematology, head and neck and reconstructive surgery, anesthesia, and critical care to develop a standardized approach for managing this unique subset of patients.     

Unraveling the Chromosomal Aberrations of Head and Neck Squamous Cell Carcinoma: A Review

Information from the genetic analysis of head and neck cancer has grown enormously in the last 20 years. The advent of high-resolution genetic analysis techniques such as microarray technology will further expand this field in the future. Here we review the data on chromosomal aberrations of head and neck squamous cell carcinoma, focusing on the data generated by comparative genomic hybridization analysis, and suggest how such findings will be taken forward over the next decade. With the search engine PUBMED, the key words "comparative genomic hybridisation," "head and neck," "oral," "hypopharyngeal," "laryngeal," and "squamous cell carcinoma" were used. Publications unavailable in English were excluded.     

Paragangliomas Arising in the Head and Neck: A Morphologic Review and Genetic Update

Seventy percent of parasympathetic paragangliomas arise in the head and neck and are nonsecretory. Awareness of the differential diagnosis based on location, overlapping morphology, and immunohistochemical profiles aids in the correct diagnosis, particularly on limited tissue samples. Moreover, 30% to 40% of head and neck paragangliomas are known to be associated with hereditary syndromes, with the succinate dehydrogenase enzyme family comprising the most frequent association. The pathologist’s role is becoming increasing critical for facilitating optimal patient care beyond the initial tissue diagnosis of paraganglioma to include screening and documenting potential hereditary tumors requiring further patient counseling and testing.     

Sclerosing Rhabdomyosarcoma: Report of a Case Arising in the Head and Neck of an Adult and Review of the Literature

Sclerosing rhabdomyosarcoma is a unique rhabdomyosarcoma variant, characterized by a prominent hyalinizing matrix. A notable pitfall is the potential for the unusual matrix and often pseudovascular growth pattern of this lesion to lead to confusion with other sarcoma types, including osteosarcoma, chondrosarcoma, and angiosarcoma. Here we report a case of sclerosing rhabdomyosarcoma arising in a 40-year old male. The tumor was centered in the pterygomaxillary fossa with extensive infiltration into adjacent structures. Fine needle aspiration yielded a preliminary diagnosis of high-grade pleomorphic undifferentiated sarcoma, for which he received neoadjuvant chemotherapy and surgical resection. Microscopic examination showed a malignant spindled to round cell neoplasm with prominent osteoid-like, hyaline stroma. Focal rhabdomyoblastic differentiation and diffuse immunoreactivity for desmin and myogenin aided in diagnosis. Nineteen months status post primary resection, the patient expired with multiple lung and bony metastases. Among 39 cases reported thus far (including the present case), there is a broad age range (0.3–79 years), with an average age at presentation of 27 years. The most commonly involved sites are the extremities (n = 19) and head and neck (n = 15). Most cases have been treated by resection, often combined with radiation and/or chemotherapy. Out of 31 cases with follow-up information provided, 6 patients developed local recurrence, 7 patients developed regional or distant metastasis, and 5 patients died of disease. Herein we discuss the ongoing controversy regarding how sclerosing rhabdomyosarcoma might best fit into existing rhabdomyosarcoma classification schemes, based upon current clinicopathologic and molecular genetic evidence.     

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure that include angiomyolipoma, lymphangioleiomyomatosis, clear cell “sugar” tumor of the lung as well as similar tumors occurring in a variety of visceral, cutaneous and soft tissue sites throughout the body. The defining histopathological features are epithelioid cells with a perivascular distribution containing clear to pale eosinophilic granular cytoplasm and a round-to-oval centrally located nucleus with an inconspicuous nucleolus. Immunohistochemically, coexpression of melanocytic (HMB-45 and/or Melan-A) and myoid markers are characteristic. In the present study, we describe three PEComas occurring in the head and neck (nasal cavity and larynx) and discuss the behavior of these distinctive tumors and review the literature of head and neck PEComas. The importance of recognizing this entity will ensure its consideration in the differential diagnosis of tumors of the head and neck with a similar morphology. The histogenesis of PEComas still remains elusive and additional cases with a prolonged follow up remain important to accurately determine the behavior of these distinctive tumors. Complete surgical excision still remains the treatment of choice for histologically benign PEComas.     

Transvaginal Bladder Neck Suspension to Cooper’s Ligament: A Review of the Literature

The aim of this paper was to review the surgical technique and clinical experience of transvaginal bladder neck suspension to Cooper’s ligament. A computerized MEDLINE search identified five English-language articles published between January 1990 and December 1998. The success rate obtained with this procedure is comparable to that obtained with the traditional Burch procedure and ranges between 86.4% and 100%. Postoperative de novo detrusor instability occurs infrequently, with an incidence ranging between 0% and 20%. Complications occur infrequently and postoperative voiding dysfunction is rare. Limited postoperative urodynamic data are available. Transvaginal suspension of the bladder neck to Cooper’s ligament combines the technique of a needle suspension with the anatomic effect of the Burch procedure.     

Vascular Anomalies of the Head and Neck: A Pediatric Overview

Vascular anomalies, further classified into vascular tumors and malformations, often involve the head and neck region of children. These entities may raise diagnostic dilemmas, as they often demonstrate heterogenous and overlapping histologic features. The aim of this paper is to provide an overview of the common vascular anomalies in the head and neck region of children. Specific entities discussed include infantile hemangioma, congenital hemangioma, tufted angioma, kaposiform hemangioendothelioma, and various vascular malformations. Clinicopathologic features and associated molecular associations are reviewed.     

Clear Cell Entities of the Head and Neck: A Selective Review of Clear Cell Tumors of the Salivary Glands

Clear cell changes may be observed in virtually any benign or malignant tumor of epithelial, mesenchymal, melanocytic and hematopoeitic derivation not be attributed to variable etiologies. In general, benign and malignant clear cell neoplasms of the head and neck are rare. They may involve various regions and may be of diverse derivations, with only 1–2% of tumors of the salivary glands, jaws and oral mucosa are primarily or almost exclusively composed of clear cells (Maiorano et al., Semin Diagn Pathol 14:203–212, 1997). This review will selectively discuss the clinicopathological features of salivary gland tumors with clear cell changes, which, at times, may pose a diagnostic challenge and dilemma.     

Sarcoidosis of the Head and Neck

Sarcoidosis is a complex disorder that often times involves the head and neck. Despite the presence of strong clinical evidence, tissue diagnosis and imaging is needed for confirmation of the disease. Although typically managed medically, when found in the sinonasal tract or intracranially, it may necessitate the intervention of a rhinologist-skull base surgeon. This article seeks to provide a comprehensive review of head and neck sarcoidosis, as this fascinating disorder often poses a diagnostic and therapeutic challenge. A brief discussion of surgical treatment for pituitary lesions is also provided. Articles from 1997 to 2013 were selected and reviewed by three researchers utilizing the most recent literature regarding sarcoidosis in the head and neck. PubMed searches were conducted using search terms such as “sarcoidosis”, “neurosarcoid”, and “extra-pulmonary sarcoid”, among many others. A large collection of articles was generated and reviewed by the team of authors, and appropriate information was extracted to compose a thorough and expansive review of the subject. 10–15 % of patients with sarcoidosis have head and neck manifestations. Sinonasal and pituitary sarcoidosis presents a diagnostic challenge owing to its non-specific symptoms. Although systemic steroid therapy is often the first time treatment, endoscopic surgery is commonly used to treat advanced pituitary sarcoidosis refractory to medical management. As tissue diagnosis and imaging is key, a multi-disciplinary team approach is advantageous. Our study collates the available literature on head and neck sarcoidosis to provide a comprehensive review of the subject. This provides helpful information to guide all practitioners involved in the care of these challenging patients, namely pathologists, radiologists, otolaryngologists, and skull base surgeons, in the workup and management of head and neck sarcoidosis.