Jean E. Lewis

Thymoma. A clinicopathologic review

The clinical and pathologic features of 283 patients with thymoma treated at the Mayo Clinic (147 female and 136 male; ages 16 years to 90 years; mean, 52 years) were examined. Forty‐six percent of the patients had myasthenia gravis and 10% had other paraneoplastic phenomena. The tumors were locally invasive at operation in 32%, including 6% with metastasis to lung or pleura. Intrathoracic recurrence was noted postoperatively in 15% of those who had total excision and distant metastasis developed in 3% of patients. Thirteen percent died of their thymomas and 16% died of myathenia. Overall 5‐year survival was 67% and 10‐year survival was 53%. Poor prognostic factors included presence of tumor‐related symptoms, large tumor size, local invasion or metastasis in initial operation, and predominantly epithelial histologic features. Although true thymomas are composed of cytologically benign elements, they show a propensity for local invasion and intrathoracic recurrence. They rarely metastasize outside the thorax.

EsthesioneuroblastomaPrognosis and Management

Forty-nine patients with esthesioneuroblastoma were treated at the Mayo Clinic between 1951 and 1990. Their clinical manifestations and treatment results were reviewed to identify possible prognostic factors. The 5-year survival rate for all patients was 69%. Tumor progression occurred in 25 patients (51%; no local control in 6 and local recurrence in 19). Metastasis was found in 15 patients (31%; regional in 10 and distant in 9). Nineteen patients died directly from metastatic or intracranial tumor extension. The pathological grade of the tumor was the most significant prognostic factor identified. The 5-year survival rate was 80% for the low-grade tumors and 40% for the high-grade tumors (P = 0.0001). Surgical treatment alone is effective for low-grade tumors if tumor-free margins can be obtained. Radiation is used for low-grade tumors when margins are close, for residual or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Recurrent tumor and regional metastasis should be treated aggressively because this approach has been shown to be worthwhile. A craniofacial resection is now the surgical procedure performed in all cases. Because recurrence can occur after 5 or even 10 years, long-term follow-up is mandatory.

Acinic cell carcinoma. Clinicopathologic review

The authors reviewed 90 cases of acinic cell carcinoma treated at the Mayo Clinic to assess long‐term follow‐up and to study features predictive of disease progression. Sixty‐three patients (55 females and 35 males) were seen for primary treatment; 27 had recurrent disease when first evaluated. Histologically, these tumors were composed of serous acinar cells and intercalated duct‐type cells. Morphologic patterns included solid, microcystic, follicular, and papillary‐cystic. All patients were followed for at least 10 years or until death. Of the primary treatment group, 44% had local recurrence, 19% had metastasis, and 25% died of disease. Disease first recurred locally up to 30 years after presentation and resulted in death after as many as 38 years. Poor prognostic features include pain or fixation; gross invasion; microscopic features of desmoplasia, atypia, or increased mitotic activity; and excision as initial treatment. Neither morphologic pattern nor cell composition was a predictive feature.

Invasive micropapillary salivary duct carcinoma : a distinct histologic variant with biologic significance

An invasive micropapillary component has been described in tumors of several organs and is nearly always associated with aggressive biologic behavior. We present 14 cases of salivary duct carcinoma (SDC) with an invasive micropapillary component (invasive micropapillary SDC) and compare the clinicopathologic findings of these cases with those of cases of conventional SDC. The mean age of the 14 patients (10 men, 4 women) was 65.8 years (range, 26–80 years). The mean size of the tumors was 2.4 cm (range, 1.3–5 cm). The parotid gland was involved in 12 patients and the submandibular gland in 2. Histologically, all tumors had an invasive micropapillary architecture admixed with features typical for SDC. Invasive micropapillary carcinoma was characterized by morula-like small cell clusters without fibrovascular cores, surrounded by a clear space. Tumor cells exhibited moderate- to high-grade nuclear features, conspicuous nucleoli, and eosinophilic cytoplasm. This component was distributed diffusely in 9 tumors and focally in 5. Angiolymphatic and perineural invasion was seen in all tumors. A residual pleomorphic adenoma was detected in four tumors. Of the 12 tumors examined, all were diffusely positive for cytokeratin 7 and epithelial membrane antigen (with a distinctive “inside-out” pattern) but negative for cytokeratin 20. Tumors were frequently immunoreactive for BRST-2 (gross cystic disease fluid protein-15) and androgen receptor protein. Aberrant expression of HER-2/neu or p53 was detected in seven tumors each. The mean Ki-67 labeling index was 33.1% (range, 6.3%–61.6%). All 14 patients with invasive micropapillary SDC had cervical or periglandular lymph node metastasis, and this value was significantly higher than for conventional SDCs. Local recurrence developed in 4 patients and distant metastatic disease in 9. Clinical follow-up (mean, 25.5 months) was available for 13 patients: 9 died of disease within 24 months after the diagnosis (mean, 17.6 months), 1 was alive with metastatic disease at 19 months, and 3 were free of disease. Overall survival of these patients with invasive micropapillary SDC was significantly shorter than that of patients with conventional SDC (n = 49) in our series (P = 0.031). Our results suggest that invasive micropapillary SDC is a distinct, aggressive variant of SDC, with a propensity for extensive lymph node metastasis and rapid disease progression.

Spindle cell carcinoma of the larynx: Review of 26 cases including DNA content and immunohistochemistry

Spindle cell carcinoma (SpCC) is uncommon, with a predilection for the upper aerodigestive tract. Its histogenesis has not been resolved, although most authors support the sarcomatoid carcinoma concept. Ploidy analysis and proliferation indices have not been reported for laryngeal SpCCs. The authors examined the pathological and clinical features of 26 patients (25 men, 1 woman; mean age, 64 years) with laryngeal SpCC treated at the Mayo Clinic from 1960 to 1990. Twenty-three tumors were examined with digital image analysis for DNA content of the spindle cell population (13 tumors had a sufficient squamous component to be analyzed separately). The glottis was involved most frequently (19 patients); 21 tumors were grossly polypoid. Twenty-three tumors were biphasic, and three were monophasic. Overall, 17 tumors (65%) showed keratin positivity in the spindle cell component. Polyclonal antikeratin (15 positive cases), 34betaE12 (15 positive), and AE1/AE3 (12 positive) were the most sensitive markers. Spindle cells were diploid in 5 tumors (22%) and nondiploid in 18 (78%); conventional squamous cell carcinoma was diploid in 4 cases and nondiploid in 9. DNA ploidy results were concordant between the two populations in 11 of 13 tumors (85%). Mean percent MIB-1 staining was 31% in the sarcomatoid component and 45% in the squamous component. In our primary treatment group of 22 patients (median follow-up, 6.4 years), 4 (18%) had local recurrence, 3 (14%) had distant metastasis, and 4 (18%) died of disease. Presence of a nondiploid spindle cell population in 78% of cases of laryngeal SpCC is interpreted as evidence of a neoplastic rather than reactive process. Keratin positivity in nearly two thirds of tumors supports the theory of epithelial origin of these tumors (sarcomatoid carcinoma).

Pediatric dermoid cysts of the head and neck

OBJECTIVE: To review the characteristics and determine treatment outcomes of pediatric dermoid cysts. STUDY DESIGN AND SETTING: Retrospective review of the presentation, diagnosis, treatment, and outcomes of all pediatric dermoid cysts of the head and neck examined between 1980 and 2002 at Mayo Clinic. RESULTS: Forty-nine patients (59% girls) had a dermoid cyst of the head and neck. The median age at diagnosis was 22 months. The most common presenting sign was a palpable mass, noted in 100% of patients. During evaluation, approximately 25 patients (51%) had imaging studies. The most common location of the cysts was periorbital (61%), followed by the neck (18%; including 1 submental cyst). Various surgical approaches were chosen. In 2 patients (4%), the dermoid cyst had an intracranial extension. Only 1 patient experienced recurrence. The median pathologic diameter of the cysts was 1.2 cm. CONCLUSIONS: Dermoid cysts are unusual neoplasms that often present in childhood, with the orbit being the area most commonly affected in the head and neck region. Imaging studies help rule out an intracranial or intraorbital extension. With complete excision, recurrence is unusual. SIGNIFICANCE: Our review will assist both primary care physicians and subspecialists in diagnosing and treating dermoid cysts.

Combined neck dissection and postoperative radiation therapy in the management of the high-risk neck: A matched-pair analysis

PURPOSE The purpose of this study was to determine the efficacy of postoperative adjuvant radiation therapy with regard to reducing the rate of recurrence in the neck, cancer-related death, and death from any cause in patients with squamous cell carcinoma of the head and neck region metastatic to neck nodes. METHODS This was a retrospective review of patients with pathologically confirmed nodal metastases who underwent neck dissection and postoperative adjuvant radiation therapy for squamous cell carcinoma of the head and neck region. Time to recurrence in the dissected area of the neck, any recurrence in the neck, cancer-related death, and death from any cause were estimated with the Kaplan-Meier method. A matched-pair analysis was performed utilizing a cohort of patients who underwent neck dissection without postoperative radiation therapy. The patients from the two cohorts were matched according to previously reported high-risk features for cancer recurrence and death. Cox hazards models for the matched pairs were used to evaluate the relative risk of subsequent recurrence in the dissected side of the neck, any neck recurrence, cancer-related death, and overall survival. MATERIALS The medical records and pathologic slides of 95 consecutive patients with pathologically confirmed nodal metastases from squamous cell carcinoma of the head and neck region who underwent neck dissection and postoperative adjuvant radiation therapy between January 1974 and December 1990 were reviewed. Previously published data from 284 patients with squamous cell carcinoma of the head and neck region treated with neck dissection alone between January 1970 and December 1980 were used for a matched-pair analysis. RESULTS The relative risks for recurrence in the dissected side of the neck, any neck recurrence (dissected neck or delayed undissected neck metastasis), cancer-related death, and death from any cause for patients treated with operation alone relative to those treated with operation and postoperative radiation were 5.82, 4.72, 2.21, and 1.67, respectively. CONCLUSION This study provides evidence that postoperative adjuvant radiation therapy for the high-risk neck can reduce the rate of recurrence within a dissected neck, delayed metastasis within an undissected neck, cancer-related death, and death from any cause.

Laryngeal amyloidosis: a clinicopathologic and immunohistochemical review.

The clinical nature of laryngeal amyloidosis has not been well established, and the natural history of the disease remains a controversial matter. To address these issues, we reviewed the clinicopathologic and immunohistochemical features of 22 cases (11 men and 11 women; mean age, 56 years) of laryngeal amyloidosis. Hoarseness was the most common symptom, and the most frequent site affected was the false vocal cords. Six patients had concomitant tracheal amyloidosis. Paraffin blocks were available for immunostaining in 20 cases. Nineteen cases were positive for amyloid P component. λ-Light chains were detected in 12 cases and κ-light chains in 5; three cases did not show definite light-chain staining. Ten patients underwent repeated operations for persistent or recurrent respiratory tract disease. One patient died of progressive tracheobronchial amyloidosis, but systemic amyloidosis did not develop in any of the patients. Laryngeal amyloidosis is a form of localized amyloidosis characterized by monoclonal light-chain deposition. Recurrent respiratory tract disease is not uncommon, but the usual clinical course is relatively benign.

Primary salivary gland-type lung cancer: spectrum of clinical presentation, histopathologic and prognostic factors.

Primary salivary‐type lung cancers are rare tumors that include adenoid cystic carcinoma (ACC) and mucoepidermoid carcinoma (MEC). The clinicopathologic profiles, symptoms on presentation, and long‐term outcomes of patients with ACC and MEC as an overall group have not been defined recently.

Merkel cell carcinoma of the head and neck: A retrospective case series

Eighty‐five percent of all Merkel cell carcinomas appear on sun‐exposed areas, with 50% to 55% occurring on the head and neck.