Proliferation index as a prognostic marker in hemangiopericytoma of the head and neck.

BACKGROUND: Hemangiopericytoma (HPC) of the head and neck is a rare neoplasm whose biologic behavior is difficult to predict by means of conventional histologic parameters. METHODS: H & E-stained sections from 12 cases of HPC were reviewed. Proliferation index was assessed using an immunoperoxidase stain for MIB-1 (Ki-67). RESULTS: The study group consisted of 4 adult men, 5 adult women, and 1 infant male. Necrosis, hypercellularity, and pleomorphism were found in 1, 5, and 6 case(s), respectively. The mitotic index per 10 high power fields varied from 0-1 to 15. Proliferation indices using MIB-1 ranged from 2.6% to 52.5%. Clinical follow-up revealed 3 cases with recurrence all possessing proliferation indices of approximately 10%. CONCLUSIONS: Standard histomorphologic features may be inadequate predictors of clinical outcome. A proliferation index of 10% or greater may indicate a more aggressive subset of HPC of the head and neck.

Sonography and MR imaging of bifid median nerve with anatomic and histologic correlation.

OBJECTIVE: Imaging of a bifid median nerve has not been previously described in the radiology literature. We present three cases of bifid median nerve. The first is a patient with carpal tunnel syndrome seen on sonography and confirmed at surgery. The other two were found among 10 cadaveric specimens and were imaged with sonography and MR imaging. Confirmation of bifid median nerve in these two specimens was obtained using anatomic and histologic correlation. CONCLUSION: Sonography and MR imaging can allow effective diagnosis and delineation of a bifid median nerve in the wrist. This diagnosis is important to make before carpal tunnel release or other wrist surgeries are performed to avoid nerve injury. Furthermore, the sonographic size criteria for diagnosing carpal tunnel syndrome in nonbifid median nerves may not be accurate in evaluating bifid median nerves.

Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.

The perivascular epithelioid cell family of tumors (PEComas), defined by their co-expression of melanocytic and muscle markers, includes angiomyolipoma, lymphangioleiomyoma, and clear cell "sugar" tumors of the lung, pancreas, and uterus. We present seven cases of a unique and previously unrecognized tumor of children and young adults, which represents a new addition to the PEComa group of tumors. Culled from three institutions over a 50-year period, all cases occurred in or immediately adjacent to the ligamentum teres and falciform ligament. Six patients were female and one male; their ages ranged from 3 to 21 years (median, 11 yrs). Tumor sizes ranged from 5 to 20 cm (median, 8 cm). All cases consisted of clear to faintly eosinophilic spindled cells arranged in fascicular and nested patterns. The cells had small but distinct nucleoli and low mitotic activity. Immunohistochemically, all cases were positive with antibodies to gp100 protein (HMB-45) and negative for S-100 protein. In three of the seven cases studied immunohistochemically, the tumors expressed smooth muscle actin, melan-A, microphthalmia transcription factor (MiTF), and myosin, but not desmin. No expression of the TSC2 gene product, tuberin, was seen in three cases. One case studied cytogenetically disclosed a t(3;10). Follow-up data, available in six of seven cases (median duration, 18 mos), showed five patients to be free of disease and one to have a radiographically presumed lung metastasis. We think these tumors comprise a new entity for which we propose the term "clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres." The differential diagnosis of these tumors includes clear cell sarcoma of tendons and aponeuroses, leiomyosarcoma, and angiomyolipoma.

Basaloid squamous cell carcinoma of the head and neck.

OBJECTIVE/HYPOTHESIS: Basaloid squamous cell carcinoma (BSCC), an uncommon tumor with predilection for the upper aerodigestive tract, is a distinct variant of squamous carcinoma, because of its unique histological features and ominous clinical behavior. This study reviews the experience in treating BSCC from two institutions. STUDY DESIGN: Retrospective. METHODS: H&E-stained sections from 20 patients with BSCC of the head and neck were reviewed and clinical follow-up was obtained for all patients. RESULTS: The study group consisted of 14 male and 6 female patients. Their ages ranged from 43 to 85 years, with a mean age of 62 years. Sites of origin included the larynx (4), tongue (3), pyriform sinus (3), nose (2), floor of mouth (2), mastoid (1), tonsil (1), epiglottis (1), nasopharynx (1), trachea (1), and palate (1). Pain was the most common presenting symptom (5 cases), followed by hoarseness and bleeding (3 cases each). Tobacco and alcohol abuse was noted in 17 patients. Treatment modalities included surgery with or without chemotherapy or radiotherapy in 13 patients, chemotherapy with irradiation in 2, chemotherapy alone in 2, and radiotherapy alone in 3. Clinical follow-up revealed no evidence of disease in 11 patients. Four were alive with disease at the time of writing and five died of disease. CONCLUSION: BSCC is a highly aggressive malignant tumor that presents in elderly patients who have a history of abuse of tobacco or alcohol, or both. Greater number of patients must be studied and compared with age-matched and stage-matched controls of conventional squamous cell carcinoma to determine whether the poor clinical outcome is related more to high-stage presentation or to the tumor's high-grade malignant cytological features.

Epstein-Barr virus in squamous carcinoma of the anterior nasal cavity.

Squamous carcinoma is the most common malignancy of the head and neck, but it rarely occurs in the nasal vestibule. Epstein-Barr virus (EBV) has been detected in and is causally linked to various head and neck tumors, particularly nasopharyngeal carcinoma. The possible role of EBV in squamous carcinoma of the anterior nasal cavity, particularly of the nasal vestibule, has not been previously investigated. Histologic sections from 17 patients with nasal vestibular squamous carcinoma were examined. Material for EBV detection by immunohistochemistry and by in situ hybridization was available in 15 of the 17 cases. The study group consisted of eight men and nine women ranging in age from 40 to 82 years (mean age, 64 years). None of the patients was of Asian descent. The squamous carcinomas were graded as well differentiated (one case), moderately differentiated (11 cases), and poorly differentiated (five cases). Fourteen patients were smokers; the history of smoking ranged from 20 to 60 pack-years. Treatment modalities included surgical resection, radiation, chemotherapy, or a combined approach. The clinical follow-up periods ranged from 7 months to 16 years. Three patients developed metastases, one of whom died of disease after 1 year. Epstein-Barr virus was not detected in any of the 15 of 17 cases tested by either immunohistochemistry or by in situ hybridization. Squamous carcinoma of the nasal vestibule is an uncommon cancer that is not causally related to EBV.

Hybrid carcinoma of the salivary gland: salivary duct adenocarcinoma adenoid cystic carcinoma.

AIMS: Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate. Their recognition is important particularly when the component tumours have different biological behaviours. The occurrence of a submandibular hybrid tumour has not been reported. METHODS AND RESULTS: We describe a case of a 36-year-old woman with a hybrid carcinoma composed of salivary duct adenocarcinoma and adenoid cystic carcinoma of the right submandibular gland. There was no evidence of a pre-existing or concurrent pleomorphic adenoma. The presence of the two components was verified by differential immunohistochemical staining using a panel of cytokeratin, vimentin, smooth muscle actin and S100. The patient subsequently developed metastases to the pelvis, lumbar, vertebra and wrist. The clinical course in this patient was consistent with the behaviour of the salivary duct adenocarcinoma component. CONCLUSIONS: The histogenesis of hybrid tumours is largely unknown, but in this case it may represent diverging differentiation of luminal tumour cells. Because some histological features of different salivary gland tumours overlap, immunohistochemistry is a valuable tool especially when used to delineate the components of a hybrid tumour.

Epithelial tumors of the lacrimal glands: a clinicopathologic study.

We report the clinicopathologic features of epithelial tumors of the lacrimal gland apparatus, which are rare and therefore represent a major challenge for diagnosis and treatment. Histologic material from 22 lesions was studied by light microscopy, histochemistry, and immunohistochemistry. A comparison with major and minor salivary gland tumors was performed to analyze the relative distribution of these tumors and to establish whether salivary glands and lacrimal gland tumors are similar or different in their pathologic appearance and clinical behavior. There were three benign pleomorphic adenomas and 19 malignant tumors. The gender distribution was equal. The ages of the patients ranged from 10 to 73 years (mean age, 46 years). Among the malignant tumors, adenoid cystic carcinoma was the most common (nine cases), followed by mucoepidermoid carcinoma (three cases). There were two cases each of malignant mixed tumor and adenocarcinoma. All mucoepidermoid carcinomas and the adenocarcinomas were histologically high grade. There also was one case each of salivary duct carcinoma, spindle cell carcinoma, and oncocytic adenocarcinoma. Of 14 patients in whom clinical follow-up was available, seven had distant metastases and four died of their disease. The only case occurring in a child was an adenoid cystic carcinoma that recurred locally after 14 years. The clinical and pathologic features of lacrimal gland tumors resemble those lesions that arise in the intraoral minor salivary glands. The greater relative proportion of malignant cases in this series probably reflects a selection bias.

Oncocytic and oncocytoid tumors of the salivary glands.

Primary pink cell tumors of the salivary glands constitute a heterogeneous group of benign and malignant lesions characterized by tumor cells with abundant eosinophilic cytoplasm. These tumors are composed predominantly of oncocytic, epidermoid, or myoepithelial cells. Tumors with a significant oncocytic component include Warthin's tumor, oncocytoma, and oncocytic carcinoma. An epidermoid component can be seen as a metaplastic change or as a true cellular constituent of a mucoepidermoid carcinoma. Myoepithelial cells may have an epithelioid character and as a consequence may impart a pink cell appearance in pleomorphic adenoma, myoepithelioma, and myoepithelial carcinoma. Usually most of these tumors are fairly distinct morphologically and do not present diagnostic dilemmas. In select circumstances, especially when dealing with a limited tissue sample, a systematic approach with an appropriate immunohistochemical panel should be used in order to arrive at a correct diagnosis. Accurate assessment is the key in the subsequent management and follow-up of these patients.

Pattern of invasion and margin assessment in patients with oral tongue cancer.

BACKGROUND: Involvement of resection margins and the pattern of tumor invasion are reported to be important predictors of local recurrence and survival in surgically treated patients. In this study we have retrospectively assessed the significance of these two prognostic factors in a relatively homogeneous patient population. Patients and Methods This study was confined to 150 previously untreated patients who had surgery for squamous carcinoma of the oral tongue between 1987 and 1993. There were 82 men and 68 women who ranged in age from 25 to 89 years (median 60 years). Glossectomy was peroral in 129, whereas 8 and 13, respectively, had a cheek flap or mandibulotomy approach. Some form of lymphadenectomy was performed in 109 (73%), and 51 patients (34%) received postoperative radiotherapy. Histologic slides from each primary tumor were reviewed to verify the margin status. In addition, the pattern of invasion was evaluated and graded from 1 to 4, varying from a consistently well-defined, "pushing" border (Grade 1) to diffuse infiltration and cellular dissociation (Grade 4). RESULTS: Intraoperative frozen section assessment of margins was accurate, whether positive or negative, in 118 of 133 patients (89%). Positive or close margins (within one high-power field) and an endophytic growth pattern were associated with a significant increase in local recurrence (p <0.003 and <0.04, respectively). With higher grades of infiltration (Grade 3 or 4; 82 patients), the tumors tended to be larger and the patients younger. Although the likelihood of nodal involvement and subsequent distant metastasis was significantly greater in those with Grade 3 or grade 4 patterns (p <0.0003 and <0. 01, respectively), there was no impact on local recurrence. Cumulative survival was similar whether or not the surgical margins were involved, but was significantly reduced when the pattern of tumor invasion was of higher grade (p <0.01). SUMMARY: Frozen section provided reasonably accurate information about margins in our patients, whether taken from the patient or the surgical specimen. Positive margins increased the likelihood of local recurrence, but did not impact on survival because subsequent surgery and/or irradiation controlled tumor recurrence in some patients. Grade 3 or 4 patterns at the tumor/host interface were associated with an increased incidence of nodal and distant metastasis, as well as a significant decrease in survival.

Giant cell tumour of the retropharynx.

AIMS: Giant cell tumours of soft tissues are usually of tenosynovial origin and are benign, slow growing neoplasms that have the capacity for local recurrence. They occur most frequently in the fingers and only rarely in the head and neck region. Their occurrence in the retropharynx has not been reported. The differential diagnoses of head and neck lesions containing giant cells are discussed. METHODS AND RESULTS: We describe a case of a retropharyngeal giant cell tumour of tenosynovial type in a 48-year-old man. The clinical, radiological and histological features are reviewed. Immunohistochemical studies point to a possible histiocytic origin based on reactivity for CD68. CONCLUSIONS: Tenosynovial giant cell tumour should be considered in the differential diagnosis of tumours arising in the retropharyngeal soft tissues.

Hodgkin's disease in the Philippines.

Hodgkin's disease (HD) varies in prevalence, morphologic findings, and association with Epstein-Barr virus (EBV) in different parts of the world. HD in the Philippines and its relationship to EBV has not been studied. We reviewed all cases of HD in the Philippines for the years 1989 to 1994, diagnosed at three large hospitals in Manila and Cavite. During this study period, 68,121 surgical specimens were accessioned, of which there were 21 cases (0.03%) of HD. There were 11 males and nine females; sex was unknown in one case. The median age was 22 yr (range, 11 to 64 yr). Thirteen cases occurred in patients less than 30 yr old, including six of 11 males and seven of nine females. The remaining cases were distributed among other age groups, with five cases in males occurring after the age of 50 yr. There were 10 cases of nodular sclerosis, nine cases of mixed cellularity, and two cases of nodular lymphocytic predominance. Nine cases were positive for EBV latent membrane protein: six of nine mixed cellularity and three of 10 nodular sclerosis. In situ hybridization confirmed the immunohistochemical results, and revealed EBV RNA predominantly in Reed-Sternberg and Hodgkin cells. A few small lymphocytes were also positive in many cases. These results suggest that HD is uncommon in the Philippines, similar to other Asian countries such as Japan. However, the age distribution and histologic subtypes of HD in the Philippines seem to be more similar to that of Western industrialized countries such as the United States. EBV was present in a subset of cases, most frequently in mixed cellularity.

Insertion of foreign bodies into the abdominal cavity. An unusual form of self-mutilation.

Self-mutilation is self-infliction of bodily injury without a conscious suicidal intent. Repeated self-inflicted abdominal stab wounds with insertion of foreign bodies into the abdominal cavity is an uncommon form of self-mutilation. We describe such a case in a 35-year-old male inmate who committed suicide by hanging. At autopsy metallic foreign bodies were found in the liver and paraduodenal soft tissue. Because of its high prevalence in the incarcerated population, self-mutilation among prisoners should be given attention. Self-mutilation should also be considered in cases of atypical behavior, psychiatric disorders, intoxication, mental retardation, and sudden death from unexplained causes.

Typhlitis in a patient with acute lymphoblastic leukemia prior to the administration of chemotherapy.

PATIENTS AND METHODS: A 3-year-old girl with acute lymphoblastic leukemia developed typhlitis immediately after diagnosis and before the institution of chemotherapy. PURPOSE: Typhlitis is a necrotizing colitis that develops in immunodeficient patients with severe neutropenia. Most patients are leukemic children who are receiving or have received chemotherapy. Typhlitis in the absence of chemotherapy is rare. We report a fatal case of typhlitis in an untreated leukemic patient. RESULTS: This case emphasizes the multifactorial pathogenesis of typhlitis, which may occur in the absence of chemotherapy. CONCLUSIONS: Typhlitis should always be considered as a potential cause of acute sepsis and abdominal pain in a leukemic patient.

Dissection and intussusception of the common carotid artery following endarterectomy.

We describe a 67-year-old man who developed a dissection of the right common carotid arterial wall with intussusception two years following endarterectomy for atherosclerosis. We suggest that the previous surgical procedure resulted in a thinned and weakened media. With a disruption of the intima, a dissection into the inner third of the media formed, and blood in the false lumen buckled the intima into the lumen of the blood vessel. Hemodynamic forces then probably played a role in the propagation of the dissection and the formation of an intussusception. Complete occlusion by thrombus followed, resulting in a massive cerebral infarct, the likely terminal event. Carotid endarterectomy rarely may be followed by dissection of the vascular wall and intussusception.