Metatypical Adenoid Cystic Carcinoma

Mathew, Elizabath; Todorovic, Emilija; Truong, Tra; Dickson, Brendan C.; Enepekides, Danny; Poon, Ian; Weinreb, Ilan

doi:10.1097/pas.0000000000001850

Cited by 16 publications

(17 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…11 These were recognized as a significant pitfall with other tumors that more typically show these morphologies, and were proven with the finding of canonical MYB::NFIB and MYBL1:: NFIB fusions in these cases. 11 Anecdotally, many head and neck experts have suggested that nonmajor gland sites may The tubular hypereosinophilia was also seen with canonical fusions and cases that were negative for fusions. A variety of morphology was seen including rhabdoid luminal cells (A) in case 15, a MYBL1::NFIB-positive laryngeal tumor; complex micropapillary and luminal-cribriform growth in case 13, a MYB::NFIB-sive maxillary tumor (B); and solid squamoid growth in case 9, a fusion negative tumor (C).…”

Section: Discussionmentioning

confidence: 84%

“…All of these findings, however, have recently been described in M-AdCC occurring in the sinonasal tract and skull base 11. These were recognized as a significant pitfall with other tumors that more typically show these morphologies, and were proven with the finding of canonical MYB::NFIB and MYBL1::NFIB fusions in these cases 11. Anecdotally, many head and neck experts have suggested that nonmajor gland sites may show more variation in morphology (personal observations of the authors).…”

Section: Discussionmentioning

confidence: 86%

“…These include macrocystic growth, squamous differentiation, extensive trabecular growth, and circumscription. All of these findings, however, have recently been described in M-AdCC occurring in the sinonasal tract and skull base 11. These were recognized as a significant pitfall with other tumors that more typically show these morphologies, and were proven with the finding of canonical MYB::NFIB and MYBL1::NFIB fusions in these cases 11.…”

Section: Discussionmentioning

confidence: 92%

“…Solid areas, which may have more atypia and mitotic activity, define the higher grade tumors. Although not specifically published, there are a number of findings that have traditionally been thought to be exclusion criteria for this entity 11. These include macrocystic growth, squamous differentiation, extensive trabecular growth, and circumscription.…”

Section: Discussionmentioning

confidence: 99%

“…For many years, we have anecdotally seen variations in AdCC morphology, mostly in nonmajor salivary gland sites. This has led to recognition of a novel subtype of AdCC, termed “metatypical adenoid cystic carcinoma” (M-AdCC) 11. Another unusual histology that we have encountered with a wide range of architecture, is striking tubular hypereosinophilia.…”

mentioning

confidence: 95%

See 4 more Smart Citations

Adenoid Cystic Carcinoma With Striking Tubular Hypereosinophilia

Weinreb

Rooper

Dickson

et al. 2023

American Journal of Surgical Pathology

Self Cite

View full text Add to dashboard Cite

The classification of salivary gland tumors is ever-evolving with new variants of tumors being described every year. Next-generation sequencing panels have helped to prove and disprove prior assumptions about tumors’ relationships to one another, and have helped refine this classification. Adenoid cystic carcinoma (AdCC) is one of the most common salivary gland malignancies and occurs at all major and minor salivary gland and seromucous gland sites. Most AdCC are predominantly myoepithelial and basaloid with variable cribriform, tubular, and solid growth. The luminal tubular elements are often less conspicuous. AdCC has largely been characterized by canonical MYB fusions, with MYB::NFIB and rarer MYBL1::NFIB. Anecdotal cases of AdCC, mostly in nonmajor salivary gland sites, have been noted to have unusual patterns, including squamous differentiation and macrocystic growth. Recently, this has led to the recognition of a subtype termed “metatypical adenoid cystic carcinoma.” Another unusual histology that we have seen with a wide range of architecture, is striking tubular hypereosinophilia. The hypereosinophilia and luminal cell prominence is in stark contrast to the vast majority of AdCC that are basaloid and myoepithelial predominant. A total of 16 cases with tubular hypereosinophilia were collected, forming morular, solid, micropapillary, and glomeruloid growth, and occasionally having rhabdoid or Paneth-like cells. They were subjected to molecular profiling demonstrating canonical MYB::NFIB (5 cases) and MYBL1::NFIB (2 cases), as well as noncanonical EWSR1::MYB (2 cases) and FUS::MYB (1 case). The remaining 6 cases had either no fusion (3 cases) or failed sequencing (3 cases). All cases were present in nonmajor salivary gland sites, with seromucous glands being the most common. These include sinonasal tract (7 cases), laryngotracheal (2 cases), external auditory canal (2 cases), nasopharynx (1 case), base of tongue (2 cases), palate (1 case), and floor of mouth (1 case). A tissue microarray of 102 conventional AdCC, including many in major salivary gland sites was examined for EWSR1 and FUS by fluorescence in situ hybridization and showed that these novel fusions were isolated to this histology and nonmajor salivary gland location. In summary, complex and striking tubular hypereosinophilia and diverse architectures are present within the spectrum of AdCC, particularly in seromucous gland sites, and may show variant EWSR1/FUS::MYB fusions.

show abstract

Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 95%

See 3 more Smart Citations

Adenoid Cystic Carcinoma With Striking Tubular Hypereosinophilia

Weinreb

Rooper

Dickson

et al. 2023

American Journal of Surgical Pathology

Self Cite

View full text Add to dashboard Cite

show abstract

Precision Medicine in the Treatment of Malignancies Involving the Ventral Skull Base: Present and Future

Ferrari

Taboni

Contro

et al. 2023

Critical Issues in Head and Neck Oncology

View full text Add to dashboard Cite

Cancers involving the ventral skull base are rare and exceedingly heterogeneous. The variety of malignant tumors that arise in the nasal cavity, paranasal sinuses, nasopharynx, and adjacent mesenchymal tissues translates into a proportionally vast spectrum of prognoses, with some histologies such as olfactory neuroblastoma being associated with rare disease-specific death to other histologies such as mucosal melanoma for which survival beyond 5 years is considered a fortunate exception. Parallel to prognosis, treatment of sinonasal cancers is complex, controversial, and deeply dependent upon the putative pretreatment diagnosis. Given their heterogeneity, cancers of the ventral skull base are particularly prone to multidisciplinary management, which is indispensable. The therapeutic options available to date for these cancers include surgery, which currently remains the mainstay of treatment in most cases, along with radiotherapy and chemotherapy. Biotherapy and immunotherapy are only anecdotally and compassionately used. For each histology, a careful selection of modalities and their timing is paramount to ensure the best chance of cure. In keeping with the principles of precision medicine, several nuances displayed by malignancies of the ventral skull base are being considered as treatment-driving characteristics. This current trend arose from the observation that a remarkable variability of behavior can be observed even within a single histology. Although evidence is lacking in this field and several potential customizations of treatment are still at a theoretical level, understanding of these cancers is rapidly evolving and practical applications of this increasing knowledge is the much-needed step forward in the management of such rare cancers. This chapter highlights the tumor characteristics that may serve as treatment-driving factors in the most relevant cancers invading the ventral skull base.

show abstract

Speicheldrüsenkarzinome – ein aktueller Überblick

Rupp

Freiberger

2022

Pathologie

View full text Add to dashboard Cite

ZusammenfassungIn den letzten Jahren hat die Charakterisierung der Speicheldrüsenkarzinome einen großen Wandel durchlebt. Morphologisch definierte Entitäten konnten zu einem Großteil auch molekular mit einem oftmals distinkten Genotyp charakterisiert werden. Der erste Teil des Artikels gibt einen Überblick über die Fortschritte der molekularen Charakteristiken des Mukoepidermoidkarzinoms, adenoid-zystischen Karzinoms, Azinuszellkarzinoms, des sekretorischen und intraduktalen Karzinoms sowie des hyalinisierenden klarzelligen Karzinoms. Der molekulare Genotyp kann dabei insbesondere bei der Klassifizierung ungewöhnlicher morphologischer Varianten von großem Nutzen sein. Rekurrente NTRK- oder RET-Genfusionen können dabei nicht nur als diagnostisches Hilfsmittel, sondern auch für eine potenzielle gezielte Therapie genutzt werden.

show abstract

Metatypical Adenoid Cystic Carcinoma

Cited by 16 publications

References 20 publications

Adenoid Cystic Carcinoma With Striking Tubular Hypereosinophilia

Adenoid Cystic Carcinoma With Striking Tubular Hypereosinophilia

Precision Medicine in the Treatment of Malignancies Involving the Ventral Skull Base: Present and Future

Speicheldrüsenkarzinome – ein aktueller Überblick

Contact Info

Product

Resources

About