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Emiltatug ledadotin (Emi-Le)

Adenoid cystic carcinoma with solid histology or high-grade transformation

Also known as XMT-1660
Manufacturer
Servier
Regulatory submission
Not announced
Launch
Not announced
Last updated
125 sources

Product information and disease descriptionPlace of product in therapyDisease description

Clinical presentation - signs and symptoms

11 evidence topics · 12 sources

Local tumor symptoms: slow-growing painless mass of the head and neck

“The prolonged asymptomatic clinical course of ACC may cause a delay in seeking medical attention.” (opens the source at this quote in a new tab)

“The most common presentation of ACC is a slow-growing, hard painless swelling in the head and the neck region, though pain and paresthesia are not uncommon as the tumor is notorious for perineural infiltration with the highest rates of perineural involvement seen in tumors arising from the parotid gland.” (opens the source at this quote in a new tab)

Neurological symptoms: pain, paresthesia, and cranial nerve palsies from perineural spread

“The clinical features depend on the site of the primary tumor and the nerve infiltrated.” (opens the source at this quote in a new tab)

“Tumors originating close to the skull base may cause ocular dysmotility, and cranial nerve palsies involving IX, X, XI, and XII. Cases of Horner's syndrome have also been reported.” (opens the source at this quote in a new tab)

“Depending on the region where the tumor originates, it can cause complications such as facial pain and paralysis, particularly if it involves the facial nerve.” (opens the source at this quote in a new tab)

“Compared to non-HGT, the HGT cohort exhibited significantly higher rates of lymph node metastasis (39.1% vs. 8.7%; P < 0.05), perineural invasion (60.9% vs. 26.1%; P < 0.05), and increased Ki-67 proliferation index (35.0% vs. 10.0%; P < 0.05).” (opens the source at this quote in a new tab)

Sinonasal and nasopharyngeal symptoms: epistaxis and nasal obstruction

“For instance, in the case of a nasopharyngeal tumor, frequent epistaxis and worsening nasal stenosis are seen along with dysfunction of the eustachian tube.” (opens the source at this quote in a new tab)

Ocular symptoms: lacrimal gland ACC

“Common presenting clinical features of lacrimal gland ACC include periorbital/periocular pain, globe dystopia, proptosis, and ‘S’-shaped ptosis.” (opens the source at this quote in a new tab)

Respiratory symptoms: tracheal primary ACC

“A total of 76 articles and 1252 cases were included in this review. The most common presenting symptom was dyspnoea (86.0%), followed by cough (58.0%).” (opens the source at this quote in a new tab)

Regional lymph node metastasis

“The incidence rate of neck metastases was 29%. The rate observed in the oral cavity is 37%, and in the major salivary glands is 19% (p = .001). The rate of occult nodal metastases was 17%.” (opens the source at this quote in a new tab)

“The high propensity for lymph node metastases suggests a role for neck dissection in patients with this rare tumor.” (opens the source at this quote in a new tab)

Distant metastatic disease: lung, bone, and liver involvement

“More than one-third of the cases progress to metastatic disease. The most common site of metastases via hematogenous spread is the lungs, followed by bone and liver.” (opens the source at this quote in a new tab)

“The lungs are the most frequent site of metastasis, followed by the bone, lymph nodes, and liver” (opens the source at this quote in a new tab)

“Watchful waiting remains an option for asymptomatic patients with metastatic ACC who have a low tumor burden due to the low response rates and indolent course of the disease.” (opens the source at this quote in a new tab)

“NOTCH1 mutations define a distinct disease phenotype characterized by solid histology, liver and bone metastasis, poor prognosis, and potential responsiveness to Notch1 inhibitors.” (opens the source at this quote in a new tab)

“Solid type ACC showed a preponderance for multiorgan metastases (17/28; 61%).” (opens the source at this quote in a new tab)

Presenting symptoms and primary sites in high-grade transformation

“The HGT group had a male-to-female ratio of 1:1.3 and a median age of 44 years (range 30–66 years). The sinonasal tract and lacrimal gland were the predominant tumor locations, accounting for 30.4% (7/23) of cases respectively.” (opens the source at this quote in a new tab)

“In the HGT group, 65.2% of participants initially displayed symptoms of dysfunction or paralysis related to the tumor location. In 13.0% (3/23) of cases, a painless mass was present, and in 17.4% (4/23), patients reported pain.” (opens the source at this quote in a new tab)

Presenting symptoms by primary site

“In the study of Nascimento et al.” (opens the source at this quote in a new tab)

“98% of patients reported a mass, 48% had pain, 30% had ulceration, and one patient had facial nerve paralysis; these symptoms had been present from 1 month to 4 years.” (opens the source at this quote in a new tab)

“The presenting symptoms of AdCC vary according to the site of disease. In major salivary glands the tumor produces a mass, and when located in the parotid, facial nerve palsy may occur; in the palate a mass is common, though ulceration or even oro-antral fistula may be seen; in the larynx dyspnea could be the first presenting symptom; in the nose and paranasal sinuses, nasal obstruction, deep facial pain, epistaxis and eye symptoms are at the forefront” (opens the source at this quote in a new tab)

Lymph node metastasis rate in high-grade transformation compared with conventional ACC

“The rate of metastasis to lymph nodes is ∼57% compared with only 5% to 25% in patients with conventional AdCC.” (opens the source at this quote in a new tab)

“The high propensity for lymph node metastasis, which is at least 5 to 10 times higher than for conventional AdCC, strongly supports neck dissection for patients with this unique histology.” (opens the source at this quote in a new tab)

Sites of metastatic sampling in a molecular profiling cohort

“Most had ACC arising from the salivary glands of the head and neck (336, 77%), but other glandular subsites were also represented. Most samples were collected from metastatic sites (283, 65%), predominately from lung (95, 34%), liver (27, 10%), and bone (22, 8%).” (opens the source at this quote in a new tab)