Primary Parotid Merkel Type Small Cell Neuroendocrine Carcinoma with Oligometastasis to the Brain and Adrenal Gland: Case Report and Review of Literature

Young, Sympascho; Oh, Justin; Bukhari, Hussam; Ng, Tony; Chau, Nichole; Tran, Eric

doi:10.1007/s12105-020-01164-w

Cited by 9 publications

(3 citation statements)

References 34 publications

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“…In the pre-avelumab era, Jacob et al reported a successful use of SRS for two metachronous brain metastases in an MCC patient who subsequently died of leptomeningeal spread [ 35 ]. Stereotactic RT showed excellent local control in another patient with limited brain metastases from a primary parotid Merkel-type small cell neuroendocrine carcinoma, which is indistinguishable from classic MCC [ 36 ]. In the latter case, avelumab would have been initiated as a salvage treatment, given the failure of chemotherapy and the limited efficacy of RT in significantly prolonging the patient’s PFS.…”

Section: Discussionmentioning

confidence: 99%

Introducing Radiotherapy in Metastatic Merkel Cell Carcinoma Patients with Limited Progression on Avelumab: An Effective Step against Primary and Secondary Immune Resistance?

Ferini¹,

Zagardo²,

Critelli

et al. 2023

JPM

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Purpose: To investigate the ability of radiotherapy (RT) to prolong progression-free survival (PFS) and to report treatment-related toxicities among oligoprogressive metastatic Merkel cell carcinoma (mMCC) patients on avelumab. Methods: We retrospectively collected clinical data on mMCC patients who underwent radiotherapy for limited progression on avelumab. Patients were categorized as primary or secondary immune refractory depending on the time of onset of resistance to immunotherapy (at the first or subsequent follow-up visits after avelumab initiation). Pre- and post-RT PFS were calculated. Overall survival (OS) from the first progression treated with RT was also reported. Radiological responses and toxicities were evaluated according to the irRECIST criteria and RTOG scoring system, respectively. Results: Eight patients, including five females, with a median age of 75 years, met our inclusion criteria. The median gross tumor and clinical target volumes at first progression on avelumab were 29.85 cc and 236.7 cc, respectively. The treatment sites included lymph node, skin, brain, and spine metastases. Four patients received more than one course of RT. Most patients were treated with palliative radiation doses (mainly 30 Gy in 3 Gy/day fractions). Two patients were treated with stereotactic RT. Five/eight patients were primary immune refractory. The objective response rate at the first post-RT assessment was 75%, whereas no local failure was reported. The median pre-RT PFS was 3 months. The pre-RT PFS was 37.5% at 6 months and 12.5% at 1 year. The median post-RT PFS was not reached. The post-RT PFS was 60% at 6 months and 1 year. The post-RT OS was 85.7% at 1 year and 64.3% at 2 years. No relevant treatment-related toxicity was observed. After a median follow-up of 18.5 months, 6/8 patients are still alive and continuing on avelumab therapy. Conclusions: Adding radiotherapy to mMCC patients with limited progression on avelumab seems to be safe and effective in prolonging the successful use of immunotherapy, regardless of the type of immune refractoriness.

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Section: Discussionmentioning

confidence: 99%

Introducing Radiotherapy in Metastatic Merkel Cell Carcinoma Patients with Limited Progression on Avelumab: An Effective Step against Primary and Secondary Immune Resistance?

Ferini¹,

Zagardo²,

Critelli

et al. 2023

JPM

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“…However, the clinical relevance of this method has not been validated. While PA usually presents as well-circumscribed, rounded hypoechoic homogenous tumors in ultrasound imaging, highly aggressive malignant tumors such as NEC and CXPA present as irregular masses with non-homogenous parenchyma and are therefore easily distinguishable from PA [35,36].…”

Section: Discussionmentioning

confidence: 99%

Novel Discovery of the Somatostatin Receptor (SSTR2) in Pleomorphic Adenomas via Immunohistochemical Analysis of Tumors of the Salivary Glands

Johnson,

Hofauer,

Wirth

et al. 2023

Cancers

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Reliable preoperative diagnosis between salivary gland tumor entities is difficult. In this monocentric retrospective study, we examined the somatostatin receptor 2 (SSTR2) status of salivary gland tumors after salivary gland tumor resection via immunohistochemistry (IHC), and stains were compared in analogy to the HER2 mamma scale. A total of 42.3% of all pleomorphic adenoma (PA) tumors (42 of 99, 95% confidence interval 32.5–52.8%) demonstrated ≥20% of cells displaying the SSTR2 as compared to just 1% of all other tumors (1/160, 95% CI 0.02–3.4%). The other tumor was a neuroendocrine carcinoma. PA had a higher intensity of SSTR2 staining, with 90.9% staining ≥ an intensity of 2 (moderate). Tumors with an intensity of SSTR2 expression equal to or greater than 2 had an 89.9% likelihood of being a PA (95% CI: 82.2–95.0%, AUC: 0.928). Only one Warthin tumor demonstrated a ‘strong’ SSTR2 staining intensity. No Warthin tumor showed a percentage of cells staining for SSTR2 above ≥20%. This result demonstrates consistent and strong expression of SSTR2 in PAs as compared to Warthin tumors, which may allow physicians to utilize radioligand-somatostatin analog PET CT/MR imaging to diagnose the PA. SSTR2 positivity, if shown to be clinically relevant, may allow peptide receptor radionuclide therapy in the future.

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“…In addition, though rare, the following tumors have specific proteins or gene alterations involving tumorigenesis: (i) nuclear protein in testis (NUT); NUT carcinoma, (ii) subfamily of ATPdependent chromatin remodeling complexes SWI/SNF (switch/sucrose non-fermentable); SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily B, member 1 (SMARCB1)-deficient high-grade transformed/dedifferentiated acinic cell carcinoma, (iii) BRAF; IPMN, (iv) neuroendocrine granules; small cell neuroendocrine carcinoma "Merkel type" (SNECM), large cell neuroendocrine carcinoma, and (v) salivary gland carcinoma with viral infection (human papillomavirus, EBV, polyomaviruses, and so on) [138][139][140][141][142][143][144][145][146][147][148][149]. These unique proteins or gene alterations might be related www.videleaf.com to tumorigenesis and, thus, may represent novel therapeutic targets.…”

Section: Other Targetable Genes and Proteinsmentioning

confidence: 99%

Histopathological Aspects of The Prognostic Factors for Salivary Gland Cancers

Nishida,

Kusaba,

Kawamura

et al. 2023

Prime Archives in Cancer Research

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Primary Parotid Merkel Type Small Cell Neuroendocrine Carcinoma with Oligometastasis to the Brain and Adrenal Gland: Case Report and Review of Literature

Cited by 9 publications

References 34 publications

Introducing Radiotherapy in Metastatic Merkel Cell Carcinoma Patients with Limited Progression on Avelumab: An Effective Step against Primary and Secondary Immune Resistance?

Introducing Radiotherapy in Metastatic Merkel Cell Carcinoma Patients with Limited Progression on Avelumab: An Effective Step against Primary and Secondary Immune Resistance?

Novel Discovery of the Somatostatin Receptor (SSTR2) in Pleomorphic Adenomas via Immunohistochemical Analysis of Tumors of the Salivary Glands

Histopathological Aspects of The Prognostic Factors for Salivary Gland Cancers

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