Polymorphous low grade adenocarcinoma with distant metastases and deletions on chromosome 6q23-qter and 11q23-qter: a case report

Hannen, E.J.M.; Bulten, Johan; Festen, J. J. M.; Wienk, S.; Wilde, P.C.M. de

doi:10.1136/jcp.53.12.942

Cited by 30 publications

(31 citation statements)

References 12 publications

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“…L'APBG récidivant montre une plus grande agressivité. Bien que rares, les métastases dans les ganglions lymphatiques régionaux peuvent se produire dans 9 % des cas et les métastases à distance sont reportées dans moins de 1 % des cas [4,5,17]. La nécessité d'un suivi régulier est justifiée par l'agressivité élevée de l'APBG en cas de récidive ou de métastase associée à un risque de transformation exceptionnelle en un néoplasme de haut grade [18,19].…”

Section: Discussionunclassified

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Adénocarcinome polymorphe de bas grade du palais mou : revue et rapport de cas

Naji

Alaoui

Yahya

2016

Med Buccale Chir Buccale

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Résumé -Introduction : L'adénocarcinome polymorphe de bas grade (APBG) est une tumeur maligne rare qui se trouve presque exclusivement située dans les glandes salivaires accessoires. Il survient fréquemment chez les patients entre 30 et 70 ans, avec un ratio femme-homme de 2/1. Il est généralement situé dans le palais dur ou mou. Observation : Il s'agissait d'une patiente de 38 ans, consultant suite à l'apparition d'une tuméfaction bleutée au niveau du palais mou droit, évoluant depuis un an, indolore et recouverte d'une muqueuse d'aspect normal. Le diagnostic évoqué était un adénome pléomorphe mais l'examen histologique après exérèse chirurgicale révélait un APBG. Une reprise des marges d'exérèse était nécessaire associée à une plastie. Discussion : Le diagnostic de l'APBG est basé principalement sur les éléments cliniques et radiologiques. L'anatomopathologie permet la confirmation et la classification de la lésion. Le traitement reste essentiellement chirurgical. Le pronostic est bon malgré un taux de récidive de l'ordre de 17 % à 24 %. Bien que rares, les métastases dans les ganglions lymphatiques régionaux peuvent se produire dans 9 % des cas. Conclusion : L'APBG est une tumeur non agressive dont le pronostic dépend de plusieurs facteurs dont le délai de consultation, d'où l'importance d'un dépistage précoce.Abstract -Polymorphous low-grade adenocarcinoma of the soft palate: review and case report. Introduction: Polymorphous low-grade adenocarcinoma (PLGA) is a rare malignant tumor that is found almost exclusively in the minor salivary glands. It frequently occurs in patients between 30 and 70 years old, with a female to male ratio of 2/1. It is usually located in the hard or soft palate. Observation: A 38-year-old female patient presented with a bluish swelling in the right side of the soft palate which had evolved for a year, which was painless and covered with a mucosa of normal appearance. The first diagnosis suggested was pleomorphic adenoma, but the histologic exam following the surgical biopsy revealed a PLGA. Surgical revision of the safety margins was necessary together with mucosal plasty. Discussion: The diagnosis of PLGA is based mainly on clinical and radiological aspects, but a histologic exam is absolutely necessary to confirm it, and for classification of the lesion. Treatment is mainly surgical. The prognosis is good, although the recurrence rate is 17% to 24%. Although seldom, metastases in regional lymph nodes may occur in 9% of cases. Conclusion: PLGA is a non-aggressive tumor whose prognosis depends on several factors including the consultation period. This highlights the importance of early detection.

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Section: Discussionunclassified

“…L'APBG a généralement un bon pronostic associé même si le taux de récidive est de 17 % à 24 %. Les métastases ganglionnaires peuvent se produire dans 9 % des cas et les métas-tases à distance sont reportées dans moins de 1 % des cas [4,5].…”

Section: Introductionunclassified

Adénocarcinome polymorphe de bas grade du palais mou : revue et rapport de cas

Naji

Alaoui

Yahya

2016

Med Buccale Chir Buccale

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“…In case 3 there was a deletion of the entire 6q arm whereas in case 5 there was an interstitial deletion of 6q22.31-6q24.3. Using low-resolution chromosomal-based CGH, Hannen and co-workers have previously described a case of PLGA with a terminal deletion of 6q23-qter (26), thus further underscoring the significance of loss of 6q in PLGA. There are 151 known genes located within the deleted 6q22.31-q24.3 segment in case 5, including the MYB oncogene and the candidate tumor suppressor gene PLAGL1.…”

Section: Discussionmentioning

confidence: 99%

Studies of genomic imbalances and the MYB-NFIB gene fusion in polymorphous low-grade adenocarcinoma of the head and neck

Persson

Fehr²,

Sundelin³

et al. 2011

Int J Oncol

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Abstract. Polymorphous low-grade adenocarcinoma (PLGA) is a malignancy predominantly originating from the minor salivary glands. The molecular events underlying the pathogenesis of PLGA is poorly understood and no recurrent genetic aberrations have so far been identified. We used genome-wide, high-resolution aCGH analysis to explore genomic imbalances in 9 cases of PLGA. Because of the well-known morphologic similarities between PLGA and adenoid cystic carcinoma (ACC) we also analyzed all tumors for expression of the recently identified ACC-associated MYB-NFIB gene fusion. aCGH analysis revealed that the PLGA genome contains comparatively few copy number alterations (CNAs). Gains/ losses of whole chromosomes or chromosome arms were more than twice as common as partial CNAs. Two cases showed gain of chromosome 8 and one case each gain of chromosome 9, loss of chromosome 22 and loss of the Y chromosome. One case showed loss of the entire 6q arm and one case an interstitial deletion of a 33-Mb segment within 6q22.1-q24.3. This region contains the MYB oncogene and the candidate tumor suppressor gene PLAGL1. RT-PCR analysis revealed that one of the 9 PLGAs expressed the ACC-associated MYB-NFIB gene fusion, illustrating the diagnostic difficulties associated with the diagnosis of these morphologically partly overlapping entities. Taken together, our findings indicate that the PLGA genome is genetically stable and contains comparatively few CNAs which is in line with the clinical observation that PLGA is a slow-growing, low-grade carcinoma with low metastatic potential.

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“…There are few cytogenetic studies of PLGA, but chromosome 12 abnormalities affecting both the q and p appear to be frequent [ 68 ]. Comparative genomic hybridisation has recently demonstrated a relative loss of material on chromosome 6q23-qter and 11q23-qter [ 46 ]. In fact, no recurrent genetic aberrations have so far been identifi ed in PLGA.…”

Section: Histopathologymentioning

confidence: 97%