Adrenocorticotropin-Secreting Pancreatoblastoma

Kletter, Gad B.; Sweetser, David A.; Wallace, Steven F.; Sawin, Robert S.; Rutledge, Joe C.; Geyer, J. Russell

doi:10.1515/jpem.2007.20.5.639

Cited by 18 publications

(5 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[5][6][7][8] Including our case, 5 of such cancers have been documented, and their clinicopathologic features are summarized in Table 4. These aggressive carcinomas were found in the pediatric age group and showed a poor outcome, as shown in Figure 3.…”

Section: Acth-secreting Accmentioning

confidence: 99%

“…In children and adolescents, ectopic ACTH secretion associated with CS has also been reported in association with pancreatic acinar cell carcinoma (ACC) and pancreatoblastoma, [5][6][7][8] rare tumor types with morphologic features sometimes overlapping those of PanNETs 9 and, for this reason, representing a diagnostic challenge for pathologists. Only 4 single cases of ACTH-secreting ACCs and pancreatoblastomas have been reported previously.…”

mentioning

confidence: 99%

See 1 more Smart Citation

ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome

Maragliano

Vanoli

Albarello

et al. 2015

American Journal of Surgical Pathology

View full text Add to dashboard Cite

Adrenocorticotropic hormone (ACTH)-secreting pancreatic neuroendocrine tumors (PanNETs), although rare, are responsible for about 15% of ectopic Cushing syndrome (CS). They represent a challenging entity because their preoperatory diagnosis is frequently difficult, and clear-cut morphologic criteria useful to differentiate them from other types of PanNETs have not been defined. Ectopic ACTH secretion associated with CS can also be rarely due to pancreatic acinar cell carcinoma (ACC) and pancreatoblastoma, rare tumor types with morphologic features sometimes overlapping those of PanNETs and, for this reason, representing a diagnostic challenge for pathologists. We herein describe the clinicopathologic and immunohistochemical features of 10 PanNETs and 1 ACC secreting ACTH and associated with CS together with an extensive review of the literature to give the reader a comprehensive overview on ACTH-producing pancreatic neoplasms. ACTH-secreting PanNETs are aggressive neoplasms with an immunohistochemical profile that partially overlaps that of pituitary corticotroph adenomas. They are generally large and well-differentiated neoplasms without distinctive histologic features but with signs of aggressiveness including vascular and perineural invasion. They are more frequent in female individuals with a mean age of 42 years. At 5 and 10 years after diagnosis, 35% and 16.2% of patients, respectively, were alive. ACTH-secreting ACCs and pancreatoblastomas are very aggressive pediatric tumors with a poor prognosis. Using an appropriate immunohistochemical panel including ACTH, β-endorphin, trypsin, and BCL10 it is possible to recognize ACTH-secreting PanNETs and to distinguish them from the very aggressive ACTH-secreting ACCs.

show abstract

Section: Acth-secreting Accmentioning

confidence: 99%

mentioning

confidence: 99%

ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome

Maragliano

Vanoli

Albarello

et al. 2015

American Journal of Surgical Pathology

View full text Add to dashboard Cite

show abstract

“…In the case reported by Li et al [ 35 ], the patient showed elevated CA19-9 and Cancer Antigen 125 levels[ 35 , 38 ]. Rarely adrenocorticotropic hormone can be secreted from the tumor[ 40 - 42 ].…”

Section: Laboratory/imaging Findingsmentioning

confidence: 99%

Blastomas of the digestive system in adults: A review

Liu,

El Jabbour,

Somma

et al. 2024

World J Gastrointest Surg

View full text Add to dashboard Cite

Blastomas, characterized by a mixture of mesenchymal, epithelial, and undifferentiated blastematous components, are rare malignant neoplasms originating from precursor blast cells. This review focuses on digestive system blastomas in adult patients, including gastroblastoma, hepatoblastoma, and pancreatoblastoma. Gastroblastoma is a biphasic, epitheliomesenchymal tumor, with only sixteen cases reported to date. In addition to the characteristic histology, metastasis-associated lung adenocarcinoma transcript 1 - glioma-associated oncogene homolog 1 gene fusion is typical, although recently novel ewing sarcoma breakpoint region 1 - c-terminal binding protein 1 and patched 1 - glioma-associated oncogene homolog 2 fusions have been described. Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty. Pancreatoblastoma, primarily a pediatric tumor, displays acinar differentiation and squamoid nests with other lines of differentiation also present, especially neuroendocrine. Diagnostic approaches for these blastomas include a combination of imaging modalities, histopathological examination, and molecular profiling. The treatment generally involves surgical resection, which may be supplemented by chemotherapy or radiotherapy in some cases. Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes, particularly in the setting of metastases. This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.

show abstract

“…This occurrence, however, is uncommon in adults 9 , 10 . A small fraction of patients may present with a concomitant para-neoplastic Cushing syndrome, due to an inappropriate secretion of ACTH 1 , 11-13 .…”

Section: Pancreatoblastomamentioning

confidence: 99%

New insights in gastrointestinal “pediatric” neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups

et al. 2022

View full text Add to dashboard Cite

Summary Pediatric solid neoplasms are rare and very different from those observed in adults. The majority of them are referred to as embryonal because they arise as a result of alterations in the processes of organogenesis or normal growth and are characterized by proliferation of primitive cells, reproducing the corresponding tissue at various stages of embryonic development. This review will focus on embryonal gastrointestinal pediatric neoplasms in adult patients, including pancreatoblastoma, hepatoblastoma, and embryonal sarcoma of the liver. Although they are classically considered pediatric neoplasms, they may (rarely) occur in adult patients. Hepatoblastoma represents the most frequent liver neoplasm in the pediatric population, followed by hepatocellular carcinoma and embryonal sarcoma of the liver; while pancreatoblastoma is the most common malignant pancreatic tumor in childhood. Both in children and adults, the mainstay of treatment is complete surgical resection, either up front or following neoadjuvant chemotherapy. Unresectable and/or metastatic neoplasms may be amenable to complete delayed surgery after neoadjuvant chemotherapy. However, these neoplasms display a more aggressive behavior and overall poorer prognosis in adults than in children, probably because they are diagnosed in later stages of diseases.

show abstract

Adrenocorticotropin-Secreting Pancreatoblastoma

Abstract: We present a 3-year-old child with Cushing's syndrome due to an ACTH-secreting metastatic pancreatoblastoma. This malignancy is a rare cause of Cushing's syndrome, particularly at pediatric age. We describe her course including the use of ketoconazole to alleviate hypercortisolemia.

Cited by 18 publications

References 18 publications

ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome

ACTH-secreting Pancreatic Neoplasms Associated With Cushing Syndrome

Blastomas of the digestive system in adults: A review

New insights in gastrointestinal “pediatric” neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups

Contact Info

Product

Resources

About