Curative high-dose reirradiation for patients with recurrent head and neck adenoid cystic carcinomas: outcomes and analysis of patterns of failure.

BACKGROUND: To investigate the outcomes of patients who underwent curative reirradiation (reRT), with intensity-modulated radiation therapy (IMRT) or proton therapy (PT) for unresectable recurrent or second primary head and neck adenoid cystic carcinoma (HNACC). METHODS: Ten patients, mostly KPS 90%, were reirradiated (3/10 with IMRT and 7/10 with PT) at a median maximum dose to the CTV of 64.2 Gy from July 2011 to November 2021. Locations at the time of reRT were mainly the sinus (4/10) and the salivary glands (including the parotid and submandibular gland, 3/10). CTCAEv5 was used to assess acute and late toxicities. Follow-up was the time between the end of reRT and the date of last news. RESULTS: The median time between the two irradiations was 53.5 months (IQR: 18-84). After a median follow-up of 26 months (range, 12.5-51.8 months), six patients had developed a locoregional recurrence (LR), of which four occurred within the previously irradiated volume. Two and three-year locoregional failure-free survival (LFFS) and overall survival (OS) were 55.6% [95%CI: 31-99.7%], and 41% [18.5-94%] and 66.7% [42-100%] and 44.4% [21.4-92.3%], respectively. LFFS and OS were significantly better in the subgroup of sinus tumors (p = .013) and the subgroup of patients re-irradiated more than two years after the first course of irradiation (p = .01). Seven patients had impairments before the start of reRT, including hearing impairment (3/10) and facial nerve impairment (3/10). The most severe late toxicities were brain necrosis (2/10), osteoradionecrosis (1/10) and vision decreased (1/10). CONCLUSION: Curative reRT for HNACC is possible for selected cases, but the LR rate in the irradiated field and the risk of severe toxicity remain high. Improved selection criteria and more carefully defined target volumes may improve outcome in these patients. A further study including larger cohort of patients would be useful to confirm these results.

Intra-mandibular adenoid cystic carcinoma.

Intra-mandibular localization of adenoid cystic carcinoma is rare. This tumor is characterized by progressive local, regional, and distant aggressiveness. We reviewed the latest data on this rare type of cancer with a small number of reported cases, alack of consensus for its treatment, and its bad prognosis.

PLAG1 and CYLD do not play a role in the tumorigenesis of adenoid cystic carcinoma.

The pleiomorphic adenoma gene 1 (PLAG1) gene is activated in a subset of pleomorphic adenomas of the salivary gland by gene fusion. Germ­line mutation in cylindromatosis (CYLD), a tumor suppressor gene, causes familial cylindromatosis and Brook­Spiegler syndrome. In the present study, aberrations in PLAG1 and CYLD were investigated in adenoid cystic carcinoma (ACC) of the salivary gland. Reverse­transcription PCR and PCR direct sequencing were performed to detect gene fusion of PLAG1 and mutation of CYLD in 34 ACC tissues. No PLAG1 fusion was detected in ACC. However, silent mutation of CYLD was detected in 2 cases of ACC, but no missense mutation was detected in ACC. These results suggest that PLAG1 and CYLD do not play a role in ACC tumorigenesis.

Carcinoma adenoide quístico / Adenoid cystic carcinoma

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[Tracheal resections]. / Trakealreseksjoner.

BACKGROUND: Tracheal resection is a valuable treatment option in patients with primary localized tumours and in selected patients with post-tracheostomy stenosis. The main symptom is dyspnoea on activity. Tracheal tumour is a rare condition with adenoid cystic carcinoma as the dominant malignant type, whereas papilloma is the most common benign lesion. MATERIAL AND METHODS: In our institution we performed eight tracheal operations from 1989 to 2002. Five patients had malignant tumours and three post-tracheostomy stenosis. The patients were reevaluated with endoscopy. RESULTS AND INTERPRETATION: Four patients with carcinomas underwent tracheal resection and direct anastomosis. One patient had postoperative irradiation due to carcinoma cells in the resection margin and died five years later. In one patient local infiltration outside the tracheal wall rendered him inoperable. Two of the three patients with benign stenoses had recidivations and underwent endoscopic dilatation and stenting. Patients with localized malignant tumours and selected patients with benign tracheal stenoses may benefit from tracheal resection. Tracheal stenosis is an important differential diagnosis in patients with airway obstruction that does not respond to pharmacological treatment.

The potential role of breast conservation surgery and adjuvant breast radiation for adenoid cystic carcinoma of the breast.

PURPOSE: Adenoid cystic carcinoma (ACC) of the breast is a rare breast cancer variant and optimal management is unclear. A review of this unusual tumour was performed at our Institution, to assess the role of breast conservation in the management of this disease. METHODS AND MATERIALS: A review of all cases of ACC of breast (1960-2000) treated at Princess Margaret Hospital (PMH) was undertaken. Information was collected on age at diagnosis, presenting features, tumour size and treatment modalities. Treatment outcomes were evaluated. RESULTS: Eighteen female and one male patient were identified. Median age at diagnosis was 58 years (range 35-76 years). Four patients had lymph-node positive disease at presentation; the single male patient presented with metastatic disease. Surgery was either a lumpectomy (10 cases) or a simple, radical or modified radical mastectomy (9 patients). Nine of 19 patients received adjuvant radiotherapy (RT). The median follow-up time was 14 years; the recurrence rate at 10 years was 31% (95% CI 7-54%) with a range in time of recurrence from 2.3 to 11.9 years. Seven recurrences were identified (4 local, 1 regional, 2 metastatic). Two of these patients developed metastatic spread and died. Six of the 19 cases went on to develop second malignancies of whom four died. Among the 18 female patients, the 10-year overall (OS), cause-specific (CSS), and relapse free survival (RFS) rates were 75, 100, and 46% respectively. CONCLUSIONS: ACC of the breast has a relatively prolonged natural history, and responds well to conservative management at presentation, with good outcome, even following local recurrence.

Adenoid cystic carcinoma associated with salivary duct cyst in the sublingual gland.

We described an extremely rare case of adenoid cystic carcinoma associated with salivary duct cyst in the sublingual gland of a 40-year-old Japanese woman. The tumor was growing from the cyst wall and almost occluded the cyst lumen. The epithelium lining the cyst lumen contained both keratin 19-positive cells and alpha-smooth muscle actin-positive cells, indicating the cyst being derived from the acinus/intercalated duct of the sublingual gland. Therefore, our case has presented for the first time a direct evidence that adenoid cystic carcinoma arises from acinus/intercalated duct.

[Primary adenoid cystic carcinoma of the nasopharynx and its relation to Epstein-Barr virus infection].

OBJECTIVE: To investigate the clinicopathologic characteristics of primary nasopharyngeal adenoid cystic carcinoma (NPACC) and its relation to Epstein-Barr virus (EBV) infection in Guangzhou where is a high-incidence area of EBV-associated nasopharyngeal carcinoma (NPC). METHODS: 17 cases of NPACC with clinical record and biopsy samples were collected in Guangzhou and their clinical manifestations were reviewed. Besides HE, Alcian blue and PAS, LSAB immunohistochemistry was performed for detecting the expression of a variety of epithelial markers, CD21 and EBV encoded LMP1. EBV encoded early RNAs (EBER) was detected by using in-situ hybridization. Nested PCR was applied for studying the presence of EBV W-fragment in tissues. RESULTS: The ratio of male to female was 7:10. The patients' age ranged from 30 to 63 years, and the median age was 46 years. 14 out of 17 tumors showed markedly local aggressive growth, presenting as T3 or T4. However, only 1 patient had metastasis of an ipsilateral cervical lymph node. The majority of neoplastic cells were basal-cell like in shape and with scanty cytoplasm and a deeply stained nucleus. Intercellular hyaline or mucinous substance was always present in between the carcinoma cells. Cribriform structure formed by the neoplastic cells could be found in 16 out of these 17 biopsies. The NPACC always express the wide-spectrum cytokeratin and the epithelium membrane antigen. Only a few or a small number of carcinoma cells showed nuclear EBER-signals in 9 cases (9/17). Concurrently, these 9 NPACCs showed a 192 bp W-fragment positive band on electrophoresis gel by nested PCR. LMP1 expression had been found in 5 out of the 9 NPACCs (55.6%) accompanying with EBER-positive carcinoma cells. The EBER-positive infiltrating lymphocytes could also be found in the stroma of 3 out of the 9 EBER-stained NPACC slides. All the tumor cells, including the EBER-positive cell of the 17 NPACCs showed no CD21 expression. CONCLUSIONS: The female is predominant over the male in development of NPACC, which often accompanied with a markedly invasive capacity at the nasopharynx and its neighboring sites. Only a small number of tumor cells, nearly a half of the studied cases were infected with EBV. Therefore, it's postulated that there seems no close relation present between NPACC and EBV infection.

Evolução clínica agressiva de carcinoma adenóide cístico sólido / Aggressive clinical course of adenoid cystic carcinoma

O carcinoma adenóide cístico (CAC) de glândula salivar é uma neoplasia maligna de crescimento lento caracterizada por grande potencial de invasão dos tecidos adjacentes além de grande propensão a recidivas e metástases. Dentre os três subtipos histológicos, o padrão sólido é reconhecidamente o de pior prognóstico. No presente trabalho, relata-se um caso de CAC do tipo sólido em paciente jovem, sexo feminino, que apresentou uma extensa lesão localizada na hemi-maxila esquerda. Através da evolução deste caso clínico, constata-se, mais uma vez, o prognóstico reservado usualmente associado ao carcinoma adenóide cístico de glândula salivar

Novel heteroplasmic frameshift and missense somatic mitochondrial DNA mutations in oral cancer of betel quid chewers.

Mitochondrial DNA (mtDNA) has been proposed to be involved in carcinogenesis because of its high susceptibility to oxidative DNA damage and limited repair mechanisms. For investigation of the potential role of somatic mtDNA mutations in the tumorigenesis of oral cancer, we screened the occurrence of mtDNA mutations by the temporal temperature gradient gel electrophoresis method. We amplified the entire mitochondrial genome by use of 32 pairs of overlapping primers, and to identify the mutations, we sequenced DNA fragments showing different banding patterns between normal and tumor mtDNA. Fourteen of eighteen (77.8%) oral carcinomas displayed somatic mtDNA mutations, with a total of 26 mutations. Among them, six were in the mRNA coding region. Three were missense mutations (C14F, H186R, T173P) in NADH dehydrogenase subunit 2, and one was a frameshift mutation, 9485delC, in cytochrome c oxidase subunit III. Eight (44%) tumors had insertion or deletion mutations in the nucleotide position 303-309 poly C region of the D-loop. Multiple large deletions were also observed. Our results demonstrate that somatic mtDNA mutations occur in oral cancer. Some missense and frameshift mutations may play an important role in the tumorigenesis of this carcinoma. More extensive biochemical and molecular studies will be necessary for determining the pathologic effect of these somatic mutations.

Carcinoma adenoide quístico de parótida. A propósito de un caso / Parotid cystic adenoid carcinoma. About a case

El carcinoma adenoide quístico constituye el 3,1 por ciento de las neoplasias malignas de la glándula parótida. Se caracteriza por la invasión perineural, la escasa afectación ganglionar y frecuentes metástasis tardías a distancia. Existen tres patrones histológicos: tubular, cribiforme y sólido, siendo este último el menos frecuente y el de peor pronóstico. El tratamiento de elección es quirúrgico.Aportamos el caso de una paciente, diagnosticada de carcinoma adenoide quístico a los 11 años de edad y que en su evolución presentó dos metástasis óseas 10 años después del tratamiento quirúrgico inicial (AU)

Tumores traqueales poco frecuentes: carcinoma adenoideo-quístico y adenoma de glándulas mucosas / Rare tracheal tumors: adenoid cystic carcinoma and mucous gland adenoma

Los tumores primarios de la tráquea son poco frecuentes y representan tan sólo el 2 por ciento de los tumores de la vía aérea superior. Se trata de un grupo heterogéneo de neoplasias de origen epitelial o conjuntivo, siendo más del 90 por ciento malignas y excepcionalmente benignas en la población adulta. El tratamiento de elección es el quirúrgico, siempre que se trate de lesiones resecables. Presentamos 2 casos intervenidos por tumores traqueales primarios infrecuentes y con connotaciones especiales. Una mujer con un carcinoma adenoideo-quístico del tercio medio traqueal con afectación de uno de los bordes de resección, que recibió radioterapia y, tras 12 años de seguimiento, no presenta recidiva, y un varón intervenido hace 11 años por un adenoma de glándulas mucosas con buena evolución, tratándose de un tumor muy raro. Realizamos una revisión bibliográfica sobre ambos tipos de tumores (AU)

Rat tumors following fractionated irradiation.

BACKGROUND: Rats are susceptible to irradiation and can develop benign and malignant tumors either spontaneously or in the field of irradiation. In the head and neck region, there are no reports available on the type of tumor after fractionated irradiation using a human therapy protocol. MATERIAL AND METHODS: We analyzed 19 tumors, in 19 rats, which developed after external X-irradiation of the left neck area in Wistar rats (2 Gy/day, monofractions, 5 days/week, total dosage 60 Gy) and compared the findings with tumors in untreated rats of the same strain. RESULTS: Tumors in the irradiation field proved to be squamous cell carcinoma or adenoid cystic carcinoma (ACC), not sarcoma. These entities were sporadically found in non-irradiated rats at a higher age. CONCLUSIONS: ACC has rarely been reported in the literature on laboratory rats. The development of this highly aggressive malignant tumor can be expected 3 months to 1 year after completion of irradiation.

[Post-traumatic adenoid cystic carcinoma of the lacrimal gland]. / Carcinome adénoïde kystique post-traumatique de la glande lacrymale.

A young 17-year-old man was injured on the external orbital canthus and developed a tumefaction which remained stable. After 2 years the tumefaction with exophthalmia and visual troubles. Radiological investigations suggested two diagnosis: Organized hematoma or a lacrimal gland tumor. Surgical exploration found an apparently benign tumor but histologically it was a cystic adenoid carcinoma.

[Intra-mandibular adenoid cystic carcinoma]. / Carcinome adénoïde kystique intra-mandibulaire.

A case of mandibular cystic adenoid carcinoma was observed in a 49-year-old man. After slow progression, the diagnosis was directed to mandibular pseudocystic tumor. The treatment was enucleoresection. Histological findings in this exceptionnal lesion led to a discussion of the radioclinical diagnosis and etiopathogenic features of adenoide cystic carcinoma. The origin of this tumor is hypothesized to be heterotopic salivary inclusion although no histologicaly proof can be provided.

Localización intramandibular de un carcinoma adenoide quístico. Repaso de la literatura / Intramandibular location of an adenoid cystic carcinoma. Review of the literature

La revisión de 13 casos descriptos en la literatura permite actualizar nuestros conocimientos a propósito de los carcinomas adenoides quísticos intramandibulares. Esto tumores, en tanto mantengan esa condición, resultan raros; el diagnóstico puede determinarse por una zona radiolúcida ubicada en la parte distal de la rama horizontal o del ángulo de la mandíbula. El diagnóstico más generalmente propuesto es el de quiste odontogénico osteolítico. La exploración quirúrgica revela un tumor sólido, desmenuzable y de poca densidad, que comprime el nervio dentario inferior. La evolución es larga e insidiosa, caracterizada principalmente por metástasis pulmonares. El tratamiento consiste en efectuar una resección ósea. Su origen aún es desconocido; puede atribuirse a inclusiones ectópicas embriológicas o a una evolución maligna de quistes odontogénicos

Localización intramandibular de un carcinoma adenoide quístico. Repaso de la literatura / Intramandibular location of an adenoid cystic carcinoma. Review of the literature

La revisión de 13 casos descriptos en la literatura permite actualizar nuestros conocimientos a propósito de los carcinomas adenoides quísticos intramandibulares. Esto tumores, en tanto mantengan esa condición, resultan raros; el diagnóstico puede determinarse por una zona radiolúcida ubicada en la parte distal de la rama horizontal o del ángulo de la mandíbula. El diagnóstico más generalmente propuesto es el de quiste odontogénico osteolítico. La exploración quirúrgica revela un tumor sólido, desmenuzable y de poca densidad, que comprime el nervio dentario inferior. La evolución es larga e insidiosa, caracterizada principalmente por metástasis pulmonares. El tratamiento consiste en efectuar una resección ósea. Su origen aún es desconocido; puede atribuirse a inclusiones ectópicas embriológicas o a una evolución maligna de quistes odontogénicos (AU)