Primary adenocarcinoma of cervical esophagus.

Most upper esophageal malignancies are squamous cell carcinomas, rarely adenocarcinomas arising from Barrett's esophagus and very rarely adenocarcinomas from heterotopic gastric mucosa without evidence of Barrett's especially in the cervical part of the esophagus. We report a case of adenocarcinoma of the polypoid type in the upper esophagus (cervical esophagus) arising from ectopic gastric mucosa, in a 60 year-old man who presented with progressive dysphagia. Accurate diagnosis by esophagogram revealed a large mass in the cervical esophagus; CAT scan showed intraluminal mass at the level of thoracic inlet, esophagogastroscopy showed a fleshy polyp (3.2cm x 3.0cm) at 20 cm from the incisors with a biopsy confirming moderately differentiated adenocarcinoma with no evidence of Barrett's esophagus. Through a left cervical approach and resection of medial third of clavicle, the tumor was removed by partial esophagectomy followed by lymph node dissection, and proved to be T1NOMO, stage I (AJCC staging 6th ed.). Post operatively, the patient received chemoradiation with no evidence of recurrence or metastasis in six years of follow up. It seems this tumor has a much better prognosis than adenocarcinomas arising from Barrett's. To our knowledge only 19 cases have been reported in literature so far.

Simultaneously presenting head and neck and lung cancer: a diagnostic and treatment dilemma.

OBJECTIVES/HYPOTHESIS: Synchronous tumors are defined as malignancies presenting within 6 months of the index tumors. A significant subset of patients present at initial evaluation with malignant tumors of both the head and neck (head and neck squamous cell carcinoma) and the lung, which are termed simultaneous primaries. The management and treatment outcomes in this cohort of patients have not been clearly defined and are the subject of the present review. STUDY DESIGN: Retrospective chart review of previously untreated patients. METHODS: From January 1974 to December 1997, a total of 2964 patients were treated for mucosal squamous cell carcinoma of the head and neck. Forty-two patients fulfilled the criteria for synchronous head and neck and lung malignancy. Of these, 27 patients had simultaneous tumors of the head and neck and the lung. This cohort of patients (n = 27) was stratified into three treatment groups. Patients in group A (n = 10) had resectable head and neck and lung primaries treated with curative intent. Group B (n = 8) was composed of patients who could have been treated with curative intent but declined and were given only palliative therapy. Patients in group C (n = 9) were candidates for only palliative treatment. RESULTS: The estimated 5-year disease-specific survival in group A was 47%, whereas patients in group B had a 5-year disease-specific survival of only 13% (P =.05). There were no survivors beyond 1 year in group C. The presence of mediastinal adenopathy in patients in group A portended poor clinical outcome. There was an estimated 5-year disease-specific survival of 51% in patients with no preoperative evidence of mediastinal adenopathy (n = 7), whereas 67% of patients with radiological evidence of mediastinal adenopathy died (two of three patients). CONCLUSION: The presence of simultaneous head and neck squamous cell carcinoma and pulmonary malignancies should not be a deterrent to aggressive surgical therapy because a potentially satisfactory outcome can be expected in these patients.

Sensate radial forearm free flaps in tongue reconstruction.

BACKGROUND: Successful rehabilitation after ablative surgery requires not only the reconstruction of 3-dimensional form but also the restoration of physiologic function. OBJECTIVE: To assess sensory recovery of reinnervated radial forearm flaps used for tongue reconstruction. PATIENTS AND METHODS: Seventeen patients, who underwent reconstruction of glossectomy defects with reinnervated radial forearm free flaps, formed the study group. Recovery of sensation was measured by both subjective and detailed objective tests 8 months after surgery. Sensory function of the flap was compared with that of the normal residual tongue or the adjacent oral mucosa and the contralateral forearm donor site. RESULTS: All patients involved in this study had tongue defects of hemiglossectomy or greater and adjacent floor of the mouth. Sensory recovery was observed in all of the 17 patients within 8 months. Detailed sensory testing showed that median static 2-point discrimination, moving 2-point discrimination, and pressure sensitivity (1.2 cm, 0.8 cm, and 3.7 psi, respectively) were subjectively greater in the innervated forearm flaps than in the contralateral forearm donor site (2.3 cm, 1.7 cm, and 4.6 psi, respectively) (P= .064) and similar to those of the normal tongue (0.9 cm, 0.5 cm, and 3.6 psi). CONCLUSIONS: In all modalities examined, sensate free flaps proved superior in sensory fidelity to the native forearm donor site and closely approached that of the normal tongue. Microsurgical reinnervation of flaps should be considered in tongue reconstruction.

Risk group-based management of differentiated thyroid carcinoma.

A number of controversies exist in the treatment of differentiated thyroid carcinoma with respect to the extent of surgery, use of radioactive iodine and post-operative thyroxine suppression. Recent recognition of prognostic factors has helped to assign patients, based on their risk profile, as being at high risk of developing recurrence. This has facilitated the development of a selective approach to therapy, thus, avoiding unnecessary treatment and reducing morbidity without compromising treatment outcome. This review attempts to evaluate the current concepts of management of differentiated thyroid carcinoma in the light of these new developments.

Clinical presentation and treatment of non-Hodgkin's lymphoma of the thyroid gland.

BACKGROUND: Non-Hodgkin's lymphoma (NHL) of the thyroid is a rare malignancy. The traditional approach to curative treatment of localized (stages I and II) NHL of the thyroid gland is surgical resection. The recent success of multimodality chemoradiotherapy suggests that surgery should be reserved for providing a tissue diagnosis or relief from acute airway obstruction. It is questionable whether this has made an impact on treatment approaches. METHODS: Retrospective chart review was conducted for all cases of localized NHL of the thyroid gland treated at Roswell Park Cancer Institute between January 1970 and January 1999. RESULTS: Ten patients (8 women, 2 men) with a mean age of 56.8 years were identified. Nine patients (90%) presented with a neck mass; seven patients (70%) had a history of Hashimoto's disease. Nine patients (90%) had extensive investigations to rule out extrathyroidal disease. All patients were treated with either a total thyroidectomy (eight patients) or a thyroid lobectomy (two patients). Nine (90%) were initially treated outside of Roswell Park Cancer Institute and referred secondarily for consideration of further therapy. Adjuvant therapy consisting of cyclophosphamide-based chemoradiotherapy was administered to nine patients. Overall survival was 80% at a mean follow-up of 8.6 years with a disease-specific survival rate of 100%. CONCLUSIONS: A review of the literature suggests that fine needle aspiration (FNA) with flow cytometry and immunohistochemistry can be used to accurately diagnose NHL of the thyroid gland. Open biopsy should be reserved for cases where this technique is not available or where the diagnosis can not be confirmed by FNA alone. Extrathyroidal NHL should be ruled out by chest x-ray, CT scan of the abdomen, and bone marrow biopsy. Further review suggests that the most efficacious therapy is systemic chemotherapy in combination with radiation for local control. Debulking surgery should be used only to provide relief from acute airway obstruction.

Sinonasal Non-Hodgkin's Lymphoma with Skull Base Involvement.

Non-Hodgkin's lymphoma (NHL) is a rare tumor of the skull base. As the incidence of primary central nervous system (CNS) lymphoma has increased, atypical presentations involving the skull or cranial base exclusively have been reported. In immunocompetent patients with no previous history or predisposing factors, the diagnosis of primary NHL of the skull base may be delayed. We present four cases of nasal and paranasal sinus NHL with both skull base and intracranial involvement in immunocompetent patients. Clinicopathologic correlation suggests that cranial base and intracranial involvement with NHL represents advanced-stage primary sinonasal disease. Surgical biopsy before definitive treatment is recommended. Radiation therapy provides local control; adjuvant chemotherapy after primary radiation therapy may be required for recurrent disease.

Iatrogenic esophagobronchial fistula arising in irradiated Barrett's esophagus.

A 47-yr-old male underwent a right upper lobectomy for stage IIB bronchoalveolar carcinoma followed by 4600 Gy of irradiation. One year later a fistula formed from an ulcerated region of Barrett's esophagus into the left main bronchus. Bronchotomy repair with onlay patch intercostal muscle flap and esophageal repair with serratus anterior muscle flap plus postoperative esophageal stent placement for stricture resulted in good functional results.

Tumour volume estimated by computed tomography as a predictive factor in carcinoma of the tongue.

This retrospective study evaluated tumour volume, estimated by computed tomography (CT), as a predictive factor in carcinoma of the tongue. Tumour volume was measured from pretreatment CT scans of 20 consecutive patients, followed up for at least 3 years, and this measurement was compared with tumour volume estimated from pathological specimens. T-stage and CT-derived tumour volume were compared with the clinical and pathological status of the nodes, and with the outcome of treatment. The measurement of tumour volume derived from CT correlated well with measurements derived from pathological examination, and tumour volume also predicted overall treatment failure. The disease-specific survival rate was 100% for patients with low-volume tumours (<13 cc) compared with 79% for those with stage T1 and T2 tumours.CT is a reliable way of measuring the volume of tumours in carcinoma of the tongue, and tumour volume is useful adjunct to the clinical tumour-node-metastases staging system.

Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors.

Malignant peripheral nerve sheath tumors of the head and neck region are uncommon and may be associated with neurofibromatosis or occur in a sporadic manner. This is a retrospective review of 17 patients with these tumors who were treated at a single institution. Analysis of clinical and pathologic factors that influenced outcome was performed. There were 9 men and 8 women. Seven patients had a history of neurofibromatosis. Radiotherapy was implicated as a possible etiologic factor in 4 patients. The neck was the most frequently involved site. Overall survival at 5 years was 52%. Survival was improved for women and for patients with low-grade tumors. Age, tumor site, and size had no impact on survival. Survival was worse for patients with neurofibromatosis than for those with the sporadic form of the disease (P = 0.02). Survival was calculated by the method of Kaplan and Meier. The significance of such results was based on results of the log rank test. Local recurrence correlated with tumor size and resection margin status. No local recurrences occurred in those patients who had negative margins of resection and received adjuvant radiotherapy. Tumor grade was predictive of the development of distant metastases. Negative margins of resection are essential for obtaining local control, and the addition of adjuvant radiotherapy may be beneficial in this group. Salvage surgery for local recurrence is possible in some patients.

Trigeminal trophic syndrome mimicking Wegener's granulomatosis. A case report with a review of the literature.

Trigeminal neuropathy with nasal ulceration, called trigeminal trophic syndrome, is an unusual complication of anesthesia in the trigeminal area. We present a case to illustrate the diagnostic and management problems that this syndrome presents.

The role of supraomohyoid neck dissection in patients with positive nodes.

BACKGROUND: Supraomohyoid neck dissection (SOHND) is currently used as a staging procedure for patients with clinically negative nodes in the neck who are at increased risk (>20%) for metastatic disease. OBJECTIVE: To assess the potential role of SOHND in patients with clinically positive nodes at levels I, II, or III. We evaluated, in particular, whether selective neck dissection in patients with clinically positive nodes results in decreased regional control and/or diminished survival. PATIENTS AND METHODS: We retrospectively reviewed the charts of all patients who underwent SOHND from January 1, 1971, to December 31, 1997. The oral cavity and oropharynx represented the primary sites in the majority of the patients. Two-year follow-up information was available on all patients. RESULTS: During the study period, 69 patients underwent 84 SOHNDs. Of the 69 patients, there were 30 patients with clinically negative nodes and 39 patients with clinically positive nodes in the neck. The overall regional control rates were 88% vs 71% for pathologically negative vs positive nodes, respectively, with or without adjuvant radiation therapy. Adjuvant radiation therapy significantly improved regional control in patients with pathologically positive nodes but not in patients with NO disease (P = .005). Similar results were noted in patients with both clinically and pathologically positive nodes. CONCLUSIONS: Supraomohyoid neck dissection in patients with pathologically positive nodes in the neck is inadequate therapy for regional control without postoperative radiation therapy. However, in patients with pathologically positive nodes in the neck, SOHND with postoperative radiation therapy can achieve regional control comparable to that of comprehensive neck dissection and postoperative radiation therapy.

Giant cell tumor of the infratemporal fossa.

Giant cell tumors are an uncommon neoplasm; most are found in the long bones, formed by endochondral ossification. This article presents a case of giant cell tumor of the infratemporal fossa, which by radiographic and clinical examination appears to have originated in the squamous portion of the temporal bone.

Patterns of nodal metastasis and surgical management of the neck in supraglottic laryngeal carcinoma.

BACKGROUND: Appropriate management of the clinically negative (N0) neck in supraglottic laryngeal cancer continues to be an area of controversy in head and neck surgery. Our treatment policy has been aggressive surgical management even in the clinically N0 neck. METHODS: Between 1971 and 1991, 104 patients had the primary diagnosis of supraglottic laryngeal cancer. Ninety of these patients received their treatment at Roswell Park Cancer Institute and are the subject of this retrospective review. RESULTS: All neoplasms included in this study were squamous cell cancers. The most common subsite involved with tumor in our series was the epiglottis, followed by the aryepiglottic folds and false cords. Supraglottic laryngectomy was performed of 29% of the cases; the remainder received total laryngectomy. Thirty-six percent of the patients had pathologic stage I/II disease, and 64% had stage III/IV. The 5-year survival rates were 100%, 81%, 73%, and 63% for stages I through IV, respectively. Fifty-seven patients had clinically N0 disease at presentation; of these 34 underwent elective neck dissection, and the remaining 23 patients were observed. Of those patients receiving neck dissection, 30% (n = 10) were found to have histologically positive disease, and of the 23 patients observed, 30% (n = 7) had histologically positive regional (neck) disease. Of the 17 clinically N0 and pathologically N+ patients, 82% (14 of 17) had involvement of level I (submandibular triangle), and 100% had involvement of level II. The incidence of bilateral disease in the clinically N0 patient was 44%. There were no local failures. CONCLUSIONS: There is a high incidence of occult regional disease even in early-stage supraglottic squamous cell carcinoma of the larynx. In the surgical management the clinically N0 neck, we presently recommend bilateral neck dissection of levels I through IV to adequately address those regions at highest risk for occult disease.

Head and neck mucosal melanoma: a 32-year review.

Because mucosal melanoma of the head or neck is uncommon, retrospective data are of value in defining its natural history, response to treatment, and patterns of recurrence. We analyzed the medical records of 28 patients who had been treated for a mucosal melanoma of the head or neck between 1961 and 1993. We found that their cumulative 5-year survival rate was 20%. Patients who had primary tumors of the nasal cavity had significantly better 5-year survival than other patients. Early stage at presentation was another predictor of a more favorable outcome. Only 2 of the 17 patients (12%) who underwent surgery died with local disease. However, 13 of these 17 surgical patients (76%) eventually died of distant metastases. There were 3 long-term (> 5 yr) survivors: 2 who were treated by surgery alone, and 1 who was treated with surgery and radiation therapy. We found that aggressive resection of the primary tumor and of any local recurrence can achieve local control in most patients with mucosal melanoma of the head or neck. Distant metastasis is the limiting factor for long-term survival.

Large, deep high-grade extremity sarcomas: when is a myocutaneous flap reconstruction necessary?

The surgical treatment of large, deep high-grade extremity soft tissue sarcomas frequently produces a significant tissue defect. In addition, the management of the surgical wound is often further complicated by preoperative radiation or adjuvant therapies. The use of either pedicled or free myocutaneous flaps allows for more rapid and predictable wound healing in this situation. Myocutaneous flaps provide well-vascularized coverage of lost tissue volume, exposed vital structures, and prosthetic reconstruction materials. When harvested from unirradiated sites, flap coverage can overcome the detrimental effects of radiation therapy and chemotherapy on postoperative wound healing. Reconstruction of the soft tissue defect may also improve patient satisfaction with aesthetic issues. The use of innervated myocutaneous flaps can even address the functionality of the extremity following resection of major muscle groups. Myocutaneous flaps are an extremely versatile option for reconstruction in the treatment of large, deep high-grade extremity soft tissue sarcomas.

Surgery versus radiation therapy as single-modality treatment of tonsillar fossa carcinoma: the Roswell Park Cancer Institute experience (1971-1991).

OBJECTIVE: To compare the efficacy and treatment outcomes in patients with tonsillar fossa cancer using surgery or radiation as a single modality therapy. METHODS: From 1971 to 1991 239 patients with oral pharyngeal cancer were treated at Roswell Park Cancer Institute. Of these patients 90 had tonsillar carcinoma. Seventy-six of these patients received either surgery (SA) (n = 56) or radiation therapy (RA) (n = 20) as single-modality therapy and are the subject of this review. All patients in the radiation arm of this review were surgical candidates who declined primary surgical therapy. RESULTS: Sixty-three percent of the SA and 80% of the RA treatment groups presented with either stage III or stage IV disease (P < or = .05). Forty-seven percent of the SA group and 52% of the RA patients had clinically positive regional disease at initial presentation. There was a predictable pattern of nodal presentation, with level II the most frequently involved region. The rate of occult metastasis was 27% and was evenly distributed between T1 and T4 disease. The overall local control rate in the SA group was 75%, compared with 60% in the RA group (P value was not significant). The disease-specific survival (all stages) was 61% in the SA group and 37% in the RA group (P < or = .05). The disease-free survival for stage III and stage IV disease in the SA group was 47% and in the RA group 27% (P < or = .05). Survival measured against clinical response to radiation therapy, in complete responders (all stages) was 83%; by contrast there were no survivors past 24 months in the partial response group (P < or = .001). CONCLUSION: The results from this study suggest that for early disease (stage I/II), surgery or radiation therapy as single-modality treatment is equally effective. For advanced disease radiation therapy is inferior to surgery as a single-modality treatment, as measured by ultimate survival and the local control of disease. There is, however, a subset of patients with advanced disease who respond to radiation therapy and whose survival is equivalent to our surgical cohort of patients.

Medullary carcinoma of the thyroid: prognostic factors and treatment recommendations.

BACKGROUND: Because medullary thyroid carcinoma accounts for only 7% of all thyroid malignancies, data to support treatment strategies are scarce. METHODS: We retrospectively reviewed treatment and outcome in 34 patients with MTC treated at Roswell Park between 1961 and 1995. Univariate analysis was performed using the variables age, sex, tumor size, N stage, and M stage. RESULTS: Median survival was 4.7 years, with 51% and 32% of patients alive at 5 and 15 years, respectively. Nodal metastases were seen in 76% and distant metastases in 67% of all patients. More than 60% of the patients with nodal metastases survived longer than 10 years. Once diagnosed with distant metastases, 90% of the patients died within 5 years. Local failure rate with lobectomy was 44%, compared to 10% after total thyroidectomy (P < .02). Age, extrathyroid extension, and M stage portend a poor outcome. Nodal status had no statistically significant impact on survival. CONCLUSION: Survival with tumors confined to the thyroid gland is independent of nodal status. Long-term survival in patients with distant metastases is rare. This study underscores the role of total thyroidectomy in the initial treatment and the need to develop effective adjuvant therapy for MTC.

Differentiated thyroid carcinoma: risk group assignment and management controversies.

In this review, we provide a framework for clinical decision-making in the treatment of differentiated thyroid cancer. The clinical discussion and treatment recommendations are relevant to an adult population (> 16 years of age). The natural history, pathogenesis, diagnostic tools, and treatment controversies in the management of this disease are explored. The roles of radioiodine therapy and thyroid-stimulating hormone (TSH) suppression and the treatment of locoregional disease are reviewed. This discussion provides a comprehensive assessment of management and treatment issues in differentiated thyroid cancer.

Patterns of failure in differentiated carcinoma of the thyroid based on risk groups.

BACKGROUND: Risk-group stratification based on prognostic factors is well established in differentiated carcinoma of the thyroid gland. Patients in the low-risk group have an excellent prognosis, whereas there is significant mortality associated with the high-risk group. The purpose of this paper is to analyze the patterns of treatment failure in the various (local, regional, distant, and associated mortality) risk groups. METHODS: In a retrospective review of a consecutive series of 1038 previously untreated patients with differentiated carcinoma of the thyroid during a period of 55 years, various prognostic factors and risk groups were analyzed. Significant prognostic factors were patient's age, presence of distant metastasis, extrathyroid extension, size, and grade of the tumor. Based on these factors, patients were divided into low- (39%), intermediate- (39%), and high- (22%) risk groups. RESULTS: The overall treatment-failure rates in the low-, intermediate-, and high-risk groups were 13%, 26%, and 50%, respectively, whereas the mortality rates in the same groups were 1%, 10%, and 33%, respectively. CONCLUSIONS: The overall incidence of recurrence rate in the low-risk group is only 13%, compared with 50% in the high-risk group. The incidence of distant metastasis in the low-risk group is only 2%, compared with 34% in the high-risk group. The understanding of the patterns of treatment failure in different risk groups reaffirms the need to direct treatment strategies based on individual risk groups and intraoperative findings.

Surgery as a single modality therapy for squamous cell carcinoma of the oral tongue.

PURPOSE: The treatment of squamous cell cancer of the oral tongue remains a challenging clinical problem. The efficacy of primary treatment with surgery versus radiation therapy for early stage disease and an adequate treatment paradigm for the clinically negative neck continues to be the subject of clinical debate. We have reviewed our experience in the treatment of oral tongue cancer with surgery as a single definitive treatment modality. PATIENTS AND METHODS: From 1971 to 1993, 79 patients with squamous cell carcinoma of the oral tongue were treated with surgery alone at Roswell Park Cancer Institute. RESULTS: Clinically, 69% of the patients presented with stage I/II disease and 31% presented with stage III/IV. Survival by pathological stage I to IV was 89%, 95%, 76%, and 65%, respectively. Surgical therapy ranged from partial to total glossectomy. There were no patients with positive margins. Local recurrence was observed in 15% of patients with close margins (< 1 cm) and 9% of patients with adequate margins (> or = 1 cm). The incidence of pathological node positive (N+) disease was 6%, 36%, 50%, and 67% for T1, T2, T3, and T4 tumors, respectively. Twenty-five percent of patients undergoing elective neck dissection were pathological N+. All pathological confirmed nodal disease was at level I or II. Of the 43 patients with clinical N0 disease, 16% subsequently developed regional recurrence, all of which were surgically salvaged. CONCLUSION: Locoregional control in patients with squamous cell carcinoma of the oral tongue can be achieved with primary surgical therapy. Adequate margins are crucial to local control. Salvage neck dissection may result in long-term survival for patients with regional relapse. Because of the high rate of occult disease (41%), we currently recommend prophylactic treatment of regional lymphatics for primary clinical disease of T2 or greater.