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[Adenoid cystic carcinoma of the lacrimal gland].

A 38-year-old female presented with a left-sided peripheral N. VI-paresis and negative neuroradiologic work-up. After one year she developed an incomplete N. III paresis, numbness of her face correlating to N. V1 and V2, reduced lacrimal secretion as well as a palpable mass at the lateral orbital rim. Magnetic resonance imaging revealed an orbital tumor extending into the cavernous sinus. Biopsy disclosed an adenoid cystic carcinoma of the lacrimal gland with basaloid, cribriform and tubular pattern. Therefore, an exenteration with postoperative radiation therapy was performed. Immunohistochemical findings included positive reactions for keratin (KL-1 and AE 1 + 3), Vimentin and Egp 34. By determination of Ki 67 the proliferative activity of the tumor was found to be 15%. Our case report demonstrates that even in absence of an exophthalmus or pain a malignant orbital tumor may be assumed as the underlying cause for a combined monolateral oculomotor and trigeminal paresis. Immunohistochemical results support the notion, that the adenoid cystic carcinoma of the lacrimal gland may arise from precursor cells of the terminal duct system.

Adult↗

Molecular typing of paired bacterial isolates from the adenoid and lateral wall of the nose in children undergoing adenoidectomy: implications in acute rhinosinusitis.

OBJECTIVE: Recent studies have suggested that the origin of bacteria that enter the lateral wall of the nose and paranasal sinuses arise from the nasopharynx. The purpose of this study was to compare the molecular biological profiles of potential pathogens found in the nasopharynx and lateral wall of the nose concomittantly in children undergoing surgery for upper respiratory tract disease. STUDY DESIGN AND SETTING: Fifty-two children undergoing adenoidectomy for either tonsillectomy or adenoidectomy (hypertrophy) or otitis media with effusion were studied. Bacterial cultures were taken from the crypts of the adenoids and from the lateral wall of the nose under endoscopic control after sterilization of the vestibule and inferior turbinate. Routine cultures of these areas were performed in the bacteriology laboratory of the Children's Hospital of Buffalo. RESULTS: Bacterial pathogens were isolated from 79% of adenoids and 46% of lateral walls of the nose. Molecular typing of pairs of nontypable Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis revealed that in 16 of 18 pairs (89%) the identical strain was present in both sites simultaneously. CONCLUSIONS: These results support the concept that when potential bacterial pathogens that may cause acute bacterial rhinosinusitis are found concomitantly in the nasopharynx and lateral wall of the nose, they are usually identical.

Acute Disease↗

Basal cell nevus syndrome concurrent with adenoid cystic carcinoma of salivary gland.

Basal cell nevus syndrome is a genetically determined disease characterized by 5 major manifestations (multiple basal cell carcinomas, jaw cysts, skeletal abnormalities, pits of the hands and feet, and ectopic calcification) and a variety of developmental anomalies. We present a case of basal cell nevus syndrome in which the patient had adenoid cystic carcinoma of the minor salivary glands develop in the soft palate resulting in distant pulmonary metastases. The patient died at the age of 44 years of respiratory complications. To our knowledge, this is the first simultaneous occurrence of basal cell nevus syndrome with adenoid cystic carcinoma of salivary gland.

Adult↗

Primary peripheral adenoid cystic carcinoma of the lung. A case report.

We report a rare case of peripheral adenoid cystic carcinoma of the lung, showing unusual pathological and clinical features, namely rapid growth, local aggressive behaviour, huge tumour size, no endobronchial component or submucosal infiltration, and a rapidly progressive clinical course. Extensive surgery resulted in considerable palliation of symptoms, but not in prolonged survival. The reported case emphasizes the malignant potential of peripheral adenoid cystic carcinoma of the lung.

Aged↗

Analysis of factors affecting long-term treatment results of adenoid cystic carcinoma of the nose and paranasal sinuses.

At Akita University Hospital (from 1971 to 1987) and at Chiba University Hospital (from 1983 to 1988), 17 patients with adenoid cystic carcinoma of the nose and paranasal sinuses were treated by en bloc tumor resection followed by primary reconstruction of the maxilla. Pre- and postoperative radiation was combined in 5 and 6 patients, respectively. Ten-year cancer-free survival rates were 59.3% in the 12 patients with maxillary sinus tumors and 50% in the 4 patients with nasal tumors. One patient with a sphenoid sinus tumor died within 5 years. Ten-year cancer-free survival was affected markedly by grade of tumor extension. Among T1N0M0 and T2N0M0 patients (of which there were 1 and 7, respectively), only 1 died of unrelated causes, and 6 patients survived cancer-free for more than 10 years. However, 4 of the 6 T3N0M0 patients died, and the cause of death was distant metastasis in 2, intracranial tumor extension in 1, and unrelated causes in 1. All 3 T4N0M0 patients died, 2 due to intracranial tumor extension and 1 of unrelated causes. The cause of death was distant metastasis in 3, local recurrence in 3, 2 of whom had intracranial tumor extension, and unrelated causes in 2. Prevention of distant metastasis and intracranial tumor extension was considered to be crucial for improving treatment results after en bloc tumor resection. Preoperative radiation was thought to decrease incidence of distant metastasis. In 5 patients who had preoperative radiation. 4 survived cancer-free for more than 10 years, and only 1 patient died of unrelated cause. However. of the 6 patients who had postoperative radiation, 2 died of distant metastasis and another 2 died of intracranial tumor extension. Of the 6 patients who did not have radiation therapy, the causes of death were local recurrence in 1, distant metastasis in 1 and unrelated causes in 1. Preoperative radiation in 5 patients showed histopathologically moderate or marked degeneration and necrosis of tumors in all patients. Although the number of patients in this study is too small to allow statistical analysis of the data, our present modality of treatment for adenoid cystic carcinoma of the nose and paranasal sinuses is routine combination of preoperative full dose radiation, en bloc tumor resection and primary reconstruction, including en bloc resection of the cranial base in selected T4 patients.

Antineoplastic Agents↗

The tubular variant of adenoid cystic carcinoma of the Bartholin's gland.

The authors report two examples of the tubular variant of adenoid cystic carcinoma of the Bartholin's gland. Each occurred in young women (both aged 34 years) and presented with a painful indurated mass, clinically thought to be of inflammatory nature. On microscopic examination, the most distinctive feature was the haphazard proliferation of bland-appearing tubular structures often lined by two cell layers. A thin, periodic acid-Schiff-positive basement membrane immunoreactive for collagen IV surrounded the tubules, but hyaline globules were virtually absent. The inner cell layer was strongly and diffusely cytokeratin positive, whereas epithelial membrane antigen reactivity was seen only along the luminal borders. Focal positivity for S100 protein identified the peripheral myoepithelial cells, which were confirmed by electron microscopy. Focal perineural invasion was seen. The histologic, ultrastructural, and immunohistochemical features of these tumors are similar to those of the tubular variant of adenoid cystic carcinoma arising in major and minor salivary glands. In one of the cases, which was followed for 6 years, the tumor metastasized, despite the low histologic grade, and the patient died. Similarities and differences between the tubular, cribriform, and solid variants and other vulvar tumors are discussed.

Adult↗

Value-based pathology: a cost-benefit analysis of the examination of routine and nonroutine tonsil and adenoid specimens.

To study the cost-effectiveness of the histologic examination of tonsil and adenoid specimens, the histologic diagnoses for all routine (2,700) and nonroutine (71) tonsil and adenoid specimens during a 10-year period were reviewed. There were 27 routine cases (1%) and 56 nonroutine cases (79%) with a diagnosis other than normal, tonsillitis, or hyperplasia. Twelve of the 27 routine cases did not have a significant clinical history, and a potentially significant pathologic diagnosis was made in only 6 cases; in none of these cases did the pathologic diagnosis affect patient care. In all nonroutine cases, the pathologic diagnosis affected patient care. The average charge per case to detect potentially significant disease in routine and nonroutine cases was $64,718 and $525, respectively. We conclude that histologic examination of nonroutine cases is cost-effective, whereas in most routine cases with adequate clinical history, histologic examination is not cost-effective.

Adenoidectomy↗

Basement membrane heparan sulfate proteoglycan (perlecan) synthesized by ACC3, adenoid cystic carcinoma cells of human salivary gland origin.

The biosynthesis of basement membrane heparan sulfate proteoglycan (HSPG), known as perlecan, in ACC3 cells established from a adenoid cystic carcinoma of the human salivary gland was studied using metabolic labeling and immunoprecipitation with discriminative antibodies specific for HSPG core protein. Treatment of immunoprecipitated HSPG with HNO2, heparitinase, and chondroitinase ABC revealed that ACC3 cells synthesized HSPG molecules composed of 470-kDa core protein and heparan sulfate but not of chondroitin sulfate. The core protein was shown to contain complex type N-linked oligosaccharides by digestion with N-glycanase and endoglycosidase H. Pulse-chase experiments showed that the mature form of HSPG was formed in the cells in 30 min and released into the medium thereafter. Degradation of HSPG was also found in the chase period of 3 h. In time course experiments, HSPG was found to be synthesized maximally at day 4 after plating, deposited in the cell layer maximally at day 6, and secreted maximally at day 8. This was also confirmed by immunofluorescence, Northern blotting, and in-situ hybridization. The results indicate that ACC3 cells synthesize, secrete and degrade basement membrane type HSPG, which is analogous to those produced by other cell types, and that the biosynthesis and secretion of HSPG in ACC3 cells are strictly regulated by the cell growth, that may be reflected in the characteristic histology of adenoid cystic carcinomas.

Basement Membrane↗

Primary cutaneous adenoid cystic carcinoma with lymph node metastasis.

We report on a case of primary cutaneous adenoid cystic carcinoma with local recurrence and lymph node metastasis. The patient was a 52-year-old Japanese woman. The initial cutaneous lesion was a skin-colored, centrally elevated, thumb-sized nodule with hair loss on the scalp in the right side of the parietal region. Three and half years after the initial combined treatment consisting of surgery and irradiation, a recurrent nodule on the left side of the parietal region of the scalp and a lymph node metastasis with perinodal tissue involvement in the left retroauricular area were discovered. In the literature on primary cutaneous adenoid cystic carcinoma, the average age in the 37 cases reported, including the present case, is 58.1 years, and 23 of the patients are women. Thirty-two percent of the carcinomas (12 cases) occur on the scalp and 16% (6 cases) on the breast. The average size of the tumors in the 24 documented cases is 3.2 cm. The local recurrence rate is 51% (19 cases). Distant metastasis to the lungs and pleura are recorded in three cases. Lymph node metastasis is recorded only in two cases including the present case. Multiple modalities including surgery, irradiation, and chemotherapy should be used for this locally aggressive and potentially metastasizing carcinoma.

Adolescent↗

Adenoid cystic carcinoma of the uterine cervix with malignant stroma. An unusual variant of carcinosarcoma?

An 80-year-old woman developed vaginal bleeding caused by a large tumor of the cervix. Histologically, the tumor proved to be composed predominantly of adenoid cystic carcinoma focally associated with squamous cell carcinoma. Of special interest was the stroma, which displayed pleomorphism, multinucleated tumor giant cells, and numerous mitoses, justifying the designation of carcinosarcoma. A single similar reported case also regarded as a carcinosarcoma described a primary cervical neoplasm in a postmenopausal woman containing an admixture of heterologous sarcoma and conventional cervical adenocarcinoma, which focally demonstrated areas of adenoid cystic carcinoma.

Aged↗

Adenoid cystic/basal cell carcinoma of the prostate: clinicopathologic findings in 19 cases.

Adenoid cystic/basal cell carcinoma (ACBCC) of the prostate has been considered to have indolent biologic potential. However, outcome data are scant, with only one documented metastasis and death. We describe clinicopathologic features of ACBCC in 19 patients and document outcome in 15. Patients ranged in age from 43 to 83 years. All but one presented with urinary obstruction. ACBCC was diagnosed by transurethral resection in 15 cases, by needle biopsy in 3 cases, and unexpected in 1 case. Four patients had concurrent acinar adenocarcinoma. Histologically, cribriform or adenoid cystic patterns predominated in 12 cases and basal cell carcinoma pattern in 7. Five cases had prominent perineural invasion. ACBCC was immunoreactive for p63 and cytokeratins 7 and 34 beta E12 but not cytokeratin 20. After diagnosis, 5 patients underwent radical prostatectomy, 2 underwent pelvic exenteration, and the rest had no treatment. ACBCC showed extraprostatic extension in 5 cases and involved the bladder margin in 3. Metastases developed in 4 (21%) patients: liver (2), lung (2), bowel (1), and corpus cavernosum (1). In 15 cases with follow-up (0.3-11.8 years), two patients died of cancer (at 1.5 and 3 years after diagnosis), 3 remain alive with cancer, and 10 have no evidence of cancer. Thus, ACBCC of the prostate is a potentially aggressive neoplasm requiring ablative therapy.

Adult↗

Giant adenoid basal cell carcinoma.

The giant adenoid basal cell carcinoma is an unusual skin tumor that has aggressive biological behavior and has been reported to exhibit geometric growth, deep invasion, and metastasis. A case of giant adenoid basal cell carcinoma is presented, with recommendations for diagnosis and treatment.

Carcinoma, Adenoid Cystic↗

Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate.

Tumors arising from minor salivary glands of the palate may exhibit an overlap of clinical and biologic features that may produce diagnostic and therapeutic dilemmas. Surgical treatment can be very different, depending on the dimensions and malignant or benign nature of the tumors, and therefore should be planned on the basis of an accurate differential diagnosis. A retrospective analysis in 24 patients with pleomorphic adenoma and adenoid cystic carcinoma of minor salivary glands of the palate was performed to investigate the accuracy of fine needle aspiration cytology (FNAC) and biopsy with histology in the preoperative diagnosis. Preoperative diagnoses obtained with FNAC and biopsy were compared with findings of the definitive histopathologic examination performed on the resected mass. Correspondence between the preoperative diagnoses determined by FNACs and the definitive histopathologic results was observed in 22 of 24 cases, whereas a complete equivalence was found with regards to histology. In the analysis, FNAC was associated with 91.6% accuracy and an error rate of 8.4% in the diagnosis of pleomorphic adenoma and adenoid cystic carcinoma of the palate. From the results of the analysis, histologic examination is still the most accurate diagnostic tool in such tumors. FNAC can be considered in tumors of the head and neck regions that are difficult to reach by means of a common biopsy.

Adenoma, Pleomorphic↗

An unusual presentation of adenoid cystic carcinoma of the lacrimal gland.

Adenoid cystic carcinoma of the lacrimal gland is a highly malignant tumor usually characterized by symptoms of less than 1 year's duration. Computed tomographic (CT) findings of adjacent bony erosion or focal tumor sclerosis are also suggestive. We present a 57-year-old female patient who manifested symptoms of an enlarging mass in the right lacrimal fossa for almost 3 years prior to the establishment of the diagnosis of adenoid cystic carcinoma. CT findings during those years were not supportive for malignancy. The treatment included en bloc resection of the mass and adjacent bone and radiotherapy.

Carcinoma, Adenoid Cystic↗

Central (intraosseous) adenoid cystic carcinoma of the mandible: report of a case with periapical involvement.

Primary intraosseous salivary gland tumors are rare, with mucopidermoid carcinoma being the most frequent histotype. The authors present a case of adenoid cystic carcinoma, located in the mandibular incisor region, associated with pain. Endodontic treatment resulted in increased pain and progressive mandibular expansion. An apicoectomy was conducted, and an intraosseous adenoid cystic carcinoma was diagnosed at histological examination. The patient was treated by wide surgical resection, and is alive and well without recurrences or distant metastases 14 yr after the original diagnosis. The case presented herein calls attention to the preoperative clinical diagnosis of periapical lesions. Radiologically, focal sclerosing osteitis, cementoblastoma, cementifying and ossifying fibroma, periapical cemental dysplasia, complex odontoma, and calcifying epithelial odontogenic tumor should be considered in the differential diagnosis. In addition the unusual occurrence of salivary gland tumors in intraosseous location stresses the importance of systematic histological examination of any tissue sample obtained after endodontic procedures.

Apicoectomy↗

Ki-67 as a prognostic marker in adenoid cystic carcinoma assessed with the monoclonal antibody MIB1 in paraffin sections.

The monoclonal antibody MIB1 recognizing the Ki-67 antigen in formalin-fixed, paraffin-embedded tissue was used to study the proliferative activity in 44 adenoid cystic carcinomas of the salivary glands. The antigen expression was compared with clinical factors, histopathological grading, and prognosis. The Ki-67 value was significantly higher in tumors from patients suffering from treatment failure than in nonfailures (P<0.001). The Ki-67 expression was also higher in tumors exhibiting areas more than 30% of the solid growth pattern and higher in sinonasal tumors than in other locations. By Cox regression analysis, Ki-67 more than 4% was the strongest prognostic indicator (P<0.005). Clinical stage and violation of surgical margins were also found to be independent significant prognostic indicators. We conclude that Ki-67 expression estimated by the use of MIB1 is a powerful tool for predicting the short-term prognosis for patients with adenoid cystic carcinoma.

Adult↗

Adenoid cystic carcinoma of the salivary glands: a review of 10 years.

A retrospective analysis was performed of 50 patients with adenoid cystic carcinoma who were seen in the Department of Radiation Oncology, University of Witwatersrand, Johannesburg, South Africa, in the past 10 years. There were 25 men and 25 women with a mean age of 52 years (age range, 21 to 88 years). Five patients had metastatic disease, and 17 had neural invasion. Thirty-four patients had surgery (11, complete; 23, microscopic residual). Sixteen patients had radiotherapy as initial management. The disease-free survival was 26%, overall survival was 29%, and local control was 30% at 10 years. Most recurrences occurred in the first 3 years. Nine patients had metastasis following treatment. The mean survival after metastasis was 15 months. Seven prognostic variables were analyzed using the log-rank test. There was no impact of age, site, type of salivary gland (major vs. minor), tumor stage, node positivity, or neural invasion on disease-free survival, overall survival, or local control. Extent of surgical resection (complete vs. microscopic residual) had a significant impact on disease-free survival and local control (P < 0.05) but no impact on overall survival (P > 0.05) because of the slow-growing nature of these tumors. Similarly, patients who had microscopic residual after surgery and were treated with radiotherapy did better than those who had biopsy and radiotherapy, although this was not significant statistically (P > 0.05). Thus, whenever possible, every attempt must be made to remove all microscopic tumor by surgery. Addition of postoperative radiotherapy with high-energy photons did not improve the locoregional control or survival in our series. There is a place for neutrons in the treatment of adenoid cystic carcinomas in advanced cases of inoperable or recurrent tumors, as a review of literature shows.

Adult↗