High Recurrence Rates of Squamous Cell Carcinoma after Mohs'' Surgery in Patients with Chronic Lymphocytic Leukemia

BACKGROUND: Cutaneous cancers exhibit a much higher incidence in patients with chronic lymphocytic leukemia than in nonleukemic patients. Squamous and basal cell carcinomas also exhibit greater subclinical tumor extension in patients with chronic lymphocytic leukemia. OBJECTIVE: The purpose of this study was to estimate and compare the recurrence rates of squamous cell carcinoma after Mohs' surgery in patients with chronic lymphocytic leukemia compared with those in controls and to evaluate differences among squamous cell carcinoma size and histologic grade. METHODS: We retrospectively assessed the clinical histories, postoperative notes, and surgical photographs of patients with chronic lymphocytic leukemia and controls matched (2:1) for age, sex, and surgical year. Both patients and controls underwent Mohs' surgery for squamous cell carcinoma of the head and neck at the Mayo Clinic between March 1988 and April 1999. RESULTS: Twenty-eight patients who underwent Mohs' surgery for 57 squamous cell carcinomas had 7 recurrences. The cumulative incidence of recurrence on a per-tumor basis was 4.3% at 1 year, 14.8% at 3 years, and 19.0% at 5 years. Squamous cell carcinoma was seven times more likely to recur in patients with chronic lymphocytic leukemia than in controls (p = .003). The distribution of tumor histologic grade was not significantly different between patients and controls (p = .39). Maximum preoperative tumor diameters were clinically similar between patients and controls (median 15 mm vs 14 mm; p = .04). CONCLUSION: The recurrence rates of squamous cell carcinoma were significantly higher in patients with chronic lymphocytic leukemia. Squamous cell carcinomas in patients with chronic lymphocytic leukemia did not exhibit a significant difference in histologic grade or clinical difference in preoperative tumor size. Close surveillance for squamous cell carcinoma recurrence is warranted in patients with chronic lymphocytic leukemia.     

Bowen's Disease (Squamous Cell Carcinoma In Situ) in Immunosuppressed Patients Treated with Imiquimod 5% Cream and a COX Inhibitor, Sulindac: Potential Applications for This Combination of Immunotherapy

BACKGROUND: Patients with chronic lymphocytic leukemia (CLL) often have a protracted course. However, all these patients are immunosuppressed and may have a high incidence of cutaneous malignancies. OBJECTIVE: To determine if combination therapy using topical imiquimod cream 5% and the oral cyclooxygenase (COX) inhibitor are useful in the therapy of squamous cell carcinoma in situ (SCC in situ)/Bowen's disease in patients with long-standing CLL. METHODS: Five CLL patients with head and neck cutaneous SCC in situ, which met criteria for Bowen's disease, were treated with topical 5% topical imiquimod cream and an oral COX inhibitor, sulindac 200 mg twice a day. RESULTS: All patients showed clinical resolution and histologic clearing of the tumors after 16 weeks of therapy. CONCLUSION: The local immune modulator, 5% imiquimod, in combination with a COX inhibitor, with its many potential antitumor effects may stimulate the innate and possibly the adaptive immune responses to clear these malignancies.     

Ulcerative balanoposthitis of the foreskin as a manifestation of chronic lymphocytic leukemia: case report and review of the literature

Ulcerative lesions of the penis have many possible etiologies, including infectious, neoplastic, traumatic, drug-induced, and autoimmune. Although the most frequent neoplasm presenting as an ulcerative penile lesion is squamous cell carcinoma, it may rarely be a manifestation of other malignancies, including those of hematolymphoid origin. We report a case of ulcerative balanoposthitis as a manifestation of chronic lymphocytic leukemia. Chronic lymphocytic leukemia and other hematolymphoid malignancies should be considered in the large differential diagnosis of nonhealing penile ulcers.     

Unilateral tonsillar enlargement

Unilateral tonsillar enlargement may result from infection, chronic inflammatory response, or neoplasm. Neoplasms that commonly produce a unilaterally enlarged tonsil include lymphomas (lymphocytic and histiocytic types) and squamous cell carcinomas. Rarer tumors include extramedullary plasmacytomas, Hodgkin's disease, leukemia, and metastatic neoplasms. Sixteen cases of unilateral tonsillar enlargement owing to causes other than squamous cell carcinoma are reviewed. When examining a patient with unilateral tonsillar enlargement, diagnosis of a neoplastic disease must be considered.     

The multiple tumor syndrome. Personal experience

The multiple tumor syndrome is an unusual pathologic condition, which consists in association of multiple malignancies in the same patient. Seven cases are discussed: two women, five men, aged 32-70 years. The period between the two neoplasias was 2-23 years (in 6 cases). In one case the two malignancies appeared concomitantly. The hematological malignancies were: multiple myeloma: 2 cases; chronic granulocytic leukemia: 2 cases; chronic lymphatic leukemia: 3 cases. In four cases, the solid tumor followed the hematological malignancy at variable periods (2 and 4 years). In other two cases, the solid tumors preceded the hematological malignancy with 2 years, 23 years respectively. The solid tumors were genital cancers, malignant melanoma, spino-cellular carcinoma, thyroid cancer, hemangiosarcoma. In a single case the second tumor was a hematological malignancy too (NHL-diffuse lymphocytic lymphoma). Possible implications of previous therapy and environmental factors are discussed.     

An unusual long-term complication of burn injury: malignant fibrous histiocytoma developed in chronic burn scar

Development of malignant tumors in chronic burn wounds is a well-known complication. These tumors are almost always squamous cell carcinomas, although other types of malignancies such as basal cell carcinoma, malignant melanoma and sarcomas can be seen rarely. There are only three previously reported cases of malignant fibrous histiocytoma developed in chronic burn scar in the literature. Two cases with malignant fibrous histiocytoma developed in chronic, badly treated burn wounds are presented. One of the tumors was multifocal and overexpression of the p53 gene was present. Both tumors were excised widely and skin grafted. Regional lymph node dissection was performed in one case. One of the patients died due to tumor recurrence and lymphatic metastases. These cases represent a very uncommon complication of burn injury and indicate the importance of the appropriate primary treatment of the burn wound.     

A Diagnosis of Chronic Lymphocytic Leukemia Prompted by Cutaneous Lymphocytic Infiltrates Present in Mohs Micrographic Surgery Frozen Sections

BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in the United States. Patients with CLL are at an increased risk for the development of second malignant neoplasms, the most common of which is cuta-neous squamous cell carcinoma (SCC). Cutaneous infiltrates of CLL have been reported in association with primary cutaneous neoplasms, including SCC, basal cell carcinoma, and actinic keratosis. The finding of a dense lymphocytic infiltrate surrounding a cutaneous neoplasm should prompt consideration of possible underlying CLL. OBJECTIVE: To review the relationship of CLL to cutaneous SCC and to raise awareness of the possible coexistence of these two neoplasms. METHODS: The case report describes a patient with a SCC of the left cheek treated with Mohs micrographic surgery. A dense lymphocytic infiltrate was noted on the frozen sections, and a complete blood count was obtained. RESULTS: A lymphocytosis was detected, leading to the diagnosis of CLL, stage 0. CONCLUSION: Patients with CLL are at an increased risk for the development of cutaneous neoplasms. An infiltrate of leukemic cells in documented CLL patients can be associated with cutaneous neoplasms such as SCC. We present a patient with no known hematologic malignancy who demonstrated a dense lymphocytic infiltrate on Mohs sections. The diagnosis of CLL was considered based on the appearance of this infiltrate. Subsequently, a new case of CLL was confirmed with appropriate testing. The finding of a dense subcutaneous infiltrate of lymphocytes on Mohs frozen sections should raise the possibility of CLL and lead to appropriate screening tests.     

Applicability of the Pittsburgh Staging System for Advanced Cutaneous Malignancy of the Temporal Bone

The objectives are to evaluate the applicability of the Pittsburgh staging system (PSS) (designed for primary temporal bone malignancies) to advanced periauricular cutaneous malignancies with temporal bone involvement and to study treatment outcomes and prognostic factors predicting recurrence-free survival. Ten patients with advanced periauricular cutaneous malignancy with temporal bone involvement were identified. Patients with primary temporal bone or parotid gland malignancies were excluded. All patients were clinically T4 at presentation by the American Joint Committee on Cancer (AJCC) staging system. Using Pittsburgh staging, six were T1 (stage I) and four were T4 (stage III). The mean follow-up was 13.6 months (3 to 24 months). Patients with basal cell carcinoma were managed with wide local excision and lateral temporal bone resection (WLE/LTBR) without adjuvant therapy. Two of three (66%) are alive and free of disease; one patient died of other causes. Treatment for squamous cell carcinoma patients involved multimodality therapy. Kaplan–Meier survival curves show a worse prognosis in terms of disease-specific survival for patients with higher-staged PSS tumors. This did not reach statistical significance. The PSS may provide additional prognostic information on advanced cutaneous malignancies of the temporal bone over the more widely used AJCC staging system. However, further prospective multicenter studies with larger sample size are required to validate our findings. Basal cell carcinoma was well controlled with WLE/LTBR alone without adjuvant therapy, whereas squamous cell carcinoma required multimodality therapy: WLE/LTBR and postoperative radiation with or without chemotherapy.     

Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum

Xeroderma pigmentosum (XP) is a rare autosomal recessive defect in DNA endonuclease activity that is associated with the development of cutaneous malignancies, at sun exposed sites, including basal cell carcinoma, squamous cell carcinoma, and melanoma. Squamous cell carcinomas are also known to target the anterior tongue. Patients sometimes develop angiosarcomas, and these invariably arise from sun-exposed skin. A biopsy was taken from a large mass arising in the anterior tongue of an 11-year-old girl with XP and a history of cutaneous basal cell carcinomas. The histopathologic findings demonstrated a high grade epithelioid neoplasm resembling a poorly differentiated squamous cell carcinoma, but the immunohistochemical profile (AE1/AE3 negative, p63 negative, CD31 positive, CD34 positive) established the diagnosis of angiosarcoma. Angiosarcoma is an XP-related tumor that usually arises in sun-exposed skin but can also arise in the oral cavity. For patients with XP who develop epithelioid neoplasms of the oral cavity, epithelioid angiosarcoma should be considered in the differential diagnosis.     

Lymphocytic Infiltrates and Subclinical Epithelial Tumor Extension in Patients With Chronic Leukemia and Solid-Organ Transplantation

BACKGROUND: Dense infiltrates in association with squamous cell carcinoma (SCC) or basal cell carcinoma (BCC) in patients with underlying chronic lymphocytic leukemia (CLL) may complicate pathologic interpretation of histologic margins. OBJECTIVE: The study was conducted to determine the frequency of identifying dense inflammatory infiltrates in frozen histologic sections during Mohs operation for BCC or SCC in patients with CLL and organ-transplant recipients, to characterize the infiltrate (reactive versus leukemic) in CLL, and to estimate the subclinical tumor extension in patients with CLL, transplant recipients, and control subjects undergoing Mohs procedure. METHODS: Frozen sections of head and neck BCC and SCC obtained during Mohs procedures in patients with CLL, organ transplant recipients, and a control group were reviewed retrospectively. Biopsy specimens of CLL with dense infiltrates were assessed with immunohistochemical stains. Subclinical tumor extension (postoperative defect size minus preoperative tumor size) was evaluated in each group. RESULTS: Dense infiltrates were found in tumors of 20 of 55 patients with CLL (36%), 1 of 8 transplant recipients (13%), and 1 of 105 controls (1%). In patients with CLL, 75% of the dense infiltrates were B-cell leukemic. Compared with controls, the mean subclinical tumor extension was larger in patients with CLL (P=0.029) and in transplant recipients (P=0.55). CONCLUSION: Dense leukemic infiltrates associated with BCC or SCC in CLL may complicate pathologic interpretation of Mohs surgical histologic margins and may be associated with larger postoperative defects relative to preoperative clinical tumor appearance. In patients with CLL, as in transplant recipients, SCC seems more likely to develop than BCC.     

Outcome of patients with early stage lung cancer

A study was conducted to evaluate the outcomes of 79 patients with early stage lung cancer diagnosed according to the following criteria. Central tumors were located in the segmental bronchi, or more proximally, and tumor invasion was limited to the bronchial wall without lymph node or distant metastases. Peripheral tumors were located distal to the subsegmental bronchi and were less than 2 cm in greatest dimension, and invasion was limited to the visceral pleura, with no lymph node or distant metastases. The 5-year survival rate was 100% for patients with peripheral type early squamous cell carcinoma, 94.6% for those with central-type early squamous cell carcinoma, and 79.3% for those with early adenocarcinoma. The 5-year survival rate for patients with central-type squamous cell carcinoma without pericartilage layer invasion was 97.0%, and that for those with T1N0M0 peripheral squamous cell carcinoma was 100.0%. To define early stage lung cancer as curable, it should be defined as T1N0M0, peripheral squamous cell carcinoma, or central squamous cell carcinoma without pericartilage layer invasion. For other histologic types, some added parameters are needed. The rate of multiple lung cancers was 10.1% and that of multiple primary malignant disease was 13.9%. Thus, careful followup of patients with early stage lung cancer should be carried out, as second malignancies in the lung and elsewhere are commonly detected.     

Concurrent FSGS and Hodgkin's lymphoma: case report and literature review on the link between nephrotic glomerulopathies and hematological malignancies

Background The link between the nephrotic syndrome (NS) and malignancy was first described in 1922. In solid tumors, the NS is most often due to membranous glomerulonephropathy, whereas in common hematological malignancies, minimal-change disease predominates. Focal segmental glomerulosclerosis (FSGS) is among the least frequently reported renal lesion associated with malignancy. Methods We report a case of the simultaneous diagnoses of FSGS and Hodgkin's lymphoma, and review the literature on various nephrotic glomerulonephropathies associated with common leukemia and lymphoma. Results Although nephrotic glomerulonephropathies rarely occur in association with acute leukemia, they have often been described in chronic lymphocytic leukemia (CLL). Membranoproliferative glomerulonephropathy and membranous glomerulonephropathy are the most common lesions observed in CLL. Nephrotic glomerulonephropathies have also been well documented among patients with lymphomas, in particular, Hodgkin's lymphoma. While minimal-change disease is most commonly found in association with Hodgkin's lymphoma, more diverse and complex renal lesions are associated with non-Hodgkin's lymphoma. FSGS remains a rare association with hematological malignancies. Conclusions Nephrotic glomerulonephropathies are not only linked to solid-organ tumors, but also to hematolo-gical malignancies. A thorough evaluation, including a physical examination for lymphadenopathy and organomegaly, as well as a hematological evaluation, must be performed in all patients presenting with nephrotic glomerulonephropathies.     

Rapid development of esophageal squamous cell carcinoma after liver transplantation for alcohol-induced cirrhosis

Liver transplant recipients have an increased risk of developing de novo malignancies. It is generally accepted that chronic alcohol abuse is a contributive factor in the pathogenesis of several malignancies, in particular, of oropharyngeal squamous cell carcinoma (SCC). Thus, patients with end-stage alcohol-induced cirrhosis could be at risk of esophageal SCC following orthotopic liver transplantation (OLT). From January 1986 to December 1997 a total of 313 patients underwent OLT for various indications. Of these patients, 72 had alcohol-related cirrhosis. Oropharyngeal and esophageal malignancies after OLT were not observed in non-alcoholic patients. In contrast, these malignancies were diagnosed in three male patients who underwent transplantation for alcohol-induced cirrhosis (incidence 4.2%). Furthermore, all patients had a history of tobacco abuse. The tumors were located in the tongue of one patient and in the esophagus of two patients. While SCC of the tongue became apparent 5 years after OLT, esophageal SCC was detected 8 and 16 months after transplantation. Shortly before transplantation, endoscopy of the esophagus had not revealed evidence of pre-malignant dysplastic lesions in any of these patients. Thus, esophageal SCC may develop rapidly in patients undergoing transplantation for alcohol-related cirrhosis with a history of tobacco abuse before liver transplantation, which warrants careful post-transplant screening of these patients.     

Cardiac operations in patients with hematologic malignancies.

OBJECTIVES: Patients with hematologic malignancies are frequently in need of major cardiac operations. Previous reports suggest an increased risk for perioperative complications in these immunodeficient patients. METHODS: Patients diagnosed with any type of hematologic malignancy who underwent open-heart surgery at our institution between 7/1996 and 6/2002 were identified. Their hospital charts were reviewed; demographics, perioperative data and outcomes were recorded. RESULTS: There were 24 patients (20 men, 4 women); mean age was 68+/-13 years (range 31-84 years). Ten patients had chronic lymphocytic leukemia, seven non-Hodgkin lymphomas, three multiple myeloma and one Hodgkin's disease, chronic myelocytic leukemia, hairy cell leukemia and cutaneous T-cell lymphoma each. The mean pre-operative duration of the hematologic disease was 6.6 years. Twenty-two patients underwent coronary artery bypass grafting (with valve replacement in three patients) and two patients had isolated valve replacement. There was one in-hospital death (4.1%). Twelve patients (50%) had a minor or major complication. Seven reoperations were required-five during the same admission (one for mediastinal bleeding, one for an expanding femoral pseudoaneurysm, one for acute cholecystitis and two for IACD/pacer insertion) and two within 30 days (one for deep sternal wound infection and one for leg wound infection). Mean post-operative stay was 8.2+/-5.8 days and mean ICU stay was 1.6+/-1.1 days. There were three late deaths-two were due to progression of the hematologic disease. The 3-year actuarial survival was 83%. CONCLUSIONS: Cardiac operations can be performed with acceptable mortality but significant morbidity rates in patients with hematologic malignancies. Bleeding and infectious complications are most frequently seen and usually lead to reoperations. These findings warrant caution during patient selection.     

Neurofibroma adjacent to the thyroid gland and a thyroid papillary carcinoma in a patient with neurofibromatosis type 1: Report of a case

Neurofibromatosis (NF) type 1, also known as von Recklinghausen’s disease, is an autosomal-dominant inherited disorder. Some tumors may develop in these patients, including optic pathway gliomas, astrocytomas, brainstem gliomas, chronic myeloid leukemia, and rhabdomyosarcoma. Patients with neurofibromatosis type 1 show also an increased risk of endocrine tumors, especially pheochromocytomas, whereas thyroid carcinoma is very rare. It is also rare for a neurofibroma to arise in the tissue neighboring the thyroid gland, and mimicking a nonfunctional thyroid nodule. This report presents a case of a neurofibroma adherent to the thyroid gland with thyroid papillary carcinoma in a 26-year-old woman with NF type 1.     

Association between urinary cytology and pathology for nontransitional cell malignancies of the urinary tract

PURPOSE: Because nontransitional cell carcinoma neoplasms of the urinary tract are rare in Western countries, we examined the association between urinary cytology and pathology evaluations for these tumors. MATERIALS AND METHODS: An institutional review board approved, retrospective review of a total of 55,946 cytology evaluations in 12,705 patients between 1992 and 2004 was performed for correlation with subsequent histopathology findings. Documented urothelial neoplasms were then correlated with previous cytology results. Nontransitional cell carcinomas were categorized as adenocarcinoma, squamous cell carcinoma and other, including small cell disease, sarcoma, melanoma or lymphoma. RESULTS: All 108 patients with cytology evaluations showing adenocarcinoma had histological evidence of cancer and 86% had adenocarcinoma in the urinary tract. All 110 patients with squamous cell carcinoma on cytology had cancer, including 47% with primary squamous cell disease. All 42 patients with other nontransitional cell carcinomas on cytology evaluation had cancer, of whom 64% had histological concordance. In a separate analysis of 70 patients who had pathologically confirmed adenocarcinoma 57% had positive prior cytology findings, of whom 19% had histological concordance. Of 85 patients with squamous cell carcinoma 81% had positive prior cytology findings, of whom 60% had histological concordance. Of 83 patients with other nontransitional cell carcinomas 70% had positive prior cytology findings, of whom 31% had histological concordance. CONCLUSIONS: In our series all patients with nontransitional cell carcinoma cytological results had cancer in the urinary tract. Thus, nontransitional cell carcinoma cytology findings mandate careful urinary tract evaluation. Concordance with histological subclassification on subsequent pathology evaluation ranges from 49% for squamous cell carcinoma to 86% for adenocarcinoma. A majority of patients with nontransitional cell carcinoma malignancies had positive prior cytology findings. However, the concordance with histological subclassification on prior cytology results ranges from 19% for adenocarcinoma to 60% for squamous cell carcinoma.     

Spindle cell (sarcomatoid) carcinomas of the larynx: a clinicopathologic study of 187 cases.

Laryngeal spindle cell (sarcomatoid) carcinomas are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. The records of 187 patients with tumors diagnosed as laryngeal spindle cell (sarcomatoid) carcinoma were retrieved from the files of the Otorhinolaryngic Tumor Registry of the Armed Forces Institute of Pathology. There were 174 men and 13 women, 35-92 years of age (average, 65.6 years). Nearly all patients experienced hoarseness (n = 165 [88%] patients) for a mean duration of 11.0 months. Patients admitted to smoking (n = 162 [87%] patients) and/or alcohol use (n = 90 [48%] patients). Most tumors were glottic (n = 132 [71%]), T1 (n = 111 [59%]), 1 and polypoid (n = 185 [99%]), with a mean tumor size of 1.8 cm. Histologically, squamous cell carcinoma (n = 157 [84%]) was noted, ulcerated, and blended with the spindle cell component, which was most frequently arranged in a storiform pattern (n = 92 [49%] tumors). Foci of benign or malignant cartilage and/or bone (n = 13 [7%]) were noted in the spindle cell component. All patients were treated with surgery (n = 90 [48%] patients) or surgery with radiation (n = 97 [52%] patients). Recurrences developed in 85 (45%) patients. Overall, T1 glottic tumors managed by complete surgical eradication had the best outcome (mean follow-up, 7.8 years).     

Metastatic Microcystic Adnexal Carcinoma in an Immunocompromised Patient

BACKGROUND: Microcystic adnexal carcinoma is an uncommon, locally aggressive cutaneous neoplasm. To date, there are only two reports of histologically proven lymph node involvement with this tumor. We describe a case of a patient with microcystic adnexal carcinoma who developed multiple local metastasis in transit with histologically proven lymph node involvement and was diagnosed with chronic lymphocytic leukemia. OBJECTIVE: To describe the details of our case and to review what is currently known about this tumor. METHODS: Mohs micrographic surgery was utilized for tumor removal. RESULTS: This patient developed multiple tumors of the scalp over the period of a 1 year which were histologically proven to be microcystic adnexal carcinoma. All tumors were noncontiguous and presented on the scalp. During the histologic analysis of the last tumor removed by Mohs micrographic surgery a lymph node was resected which revealed infiltrative microcystic adnexal carcinoma. CONCLUSIONS: We present the case of an immunocompromised patient treated for microcystic adnexal carcinoma with Mohs micrographic surgery who proceeded to develop local metastasis in transit.     

A high association of upper urinary tract transitional cell carcinoma with nonfunctioning kidney caused by stone disease in Taiwan

INTRODUCTION: Although many studies have reported that upper urinary tract stones in patients with nonfunctioning kidneys are associated with malignancy, the incidence and frequency of the tumor cell type remain unclear. Therefore, we aimed to investigate the percentage of malignancies present in patients with nonfunctional kidneys who underwent nephrectomy. MATERIALS AND METHODS: From July 1995 to December 2005, we reviewed a total of 47 patients who underwent nephrectomy to treat a nonfunctional kidney due to urolithiasis with clinical symptoms such as complicated chronic infection. Pathology revealed malignancies in 24 patients. Relationships between clinical presentations and malignancy were analyzed. RESULTS: Malignancy was diagnosed in 24 patients after nephrectomy. Image studies revealed possible malignancy in only 7 patients before surgery. A high incidence (17/24) of transitional cell carcinoma was noted. The remaining malignancies included 5 renal cell carcinomas, 1 squamous cell carcinoma and 1 epidermoid carcinoma. There were 5 deaths during the follow-up interval. CONCLUSIONS: There is a high incidence of malignancy associated with nonfunctional kidney caused by stone disease. We suggest careful examination of the pathological specimens from nephrectomy for nonfunctional kidney regardless of clinical manifestations.     

Primary Squamous Cell Carcinoma of the Parotid Gland: The Importance of Correct Histological Diagnosis

Background: Primary squamous cell carcinoma of the parotid is an uncommon, aggressive malignancy with a poor prognosis. The diagnosis is made after excluding metastasis from other sites in the head and neck or other primary malignancies of the parotid.Methods: Tumor registry data from 1974 to 1994 were reviewed at three University of Louisville- affiliated hospitals. Of 370 parotid tumors, 40 (11%) were initially classified as squamous cell carcinoma of the parotid. Chart review and histological specimen re-examination were conducted to confirm diagnosis.Results: Only 8 (2%) of the 370 cases, were considered true primary squamous cell carcinoma of the parotid. Patients with metastases to the parotid from primary sites within the upper aerodigestive tract or skin of the head and neck region and high-grade mucoepidermoid carcinoma of the parotid were excluded. Facial nerve dysfunction was a presenting complaint in three patients. Two patients presented with American Joint Committee on Cancer (AJC) clinical stage III disease and six with AJC stage IV disease. All patients were treated with total parotidectomy and radiotherapy. One patient (12%) is alive and free of disease. Median survival was 13 months (range, 11 months–7 years).Conclusions: Primary squamous cell carcinoma of the parotid is uncommon, occurring in 2% of parotid neoplasms at our institution. This is an aggressive malignancy, usually presenting in advanced stage and with facial nerve involvement or cervical metastases. Prognosis is poor even with radical surgery and adjunctive radiotherapy. Careful clinical and histological review is necessary to differentiate primary squamous cancer of the parotid from metastases or other primary parotid malignancy.