Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome

Tehseen, Sarah; Rapkin, Louis; Schemankewitz, Erwin; Magliocca, Joseph F.; Romero, René

doi:10.1111/petr.13000

Cited by 12 publications

(12 citation statements)

References 15 publications

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“…Calcifying Nested Stromal Epithelial Tumor (CNSET) is an exceptionally uncommon primary hepatic tumor that occurs in children and young adults, with only about 40 cases reported in the literature [36]. While localized disease is often effectively cured with surgical resection, recurrent and de novo metastatic disease demonstrates chemotherapy resistance and there are no proven therapeutic options [37][38][39][40].…”

Section: Pharmacotypes Identification For Clinical Trial Designmentioning

confidence: 99%

Pre-clinical validation of an RNA-based precision oncology platform for patient-therapy alignment in a diverse set of human malignancies resistant to standard treatments

Mundi¹,

Cruz²,

Grunn³

et al. 2021

Preprint

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Predicting tumor sensitivity to antineoplastics remains an elusive challenge, with no methods demonstrating predictive power. Joint analysis of tumors-from patients with distinct malignancies who had progressed on multiple lines of therapy-and drug perturbation transcriptional profiles predicted sensitivity to 28 of 350 drugs, 26 of which (93%) were confirmed in low-passage, patient-derived xenograft (PDX) models. Drugs were prioritized based on their ability to either invert the activity of individual Master Regulator proteins, with available high-affinity inhibitors, or of the modules they comprise (Tumor-Checkpoints), based on de novo mechanism of action analysis. Of 138 PDX mice enrolled in 16 single and 18 multi-protein treatment arms, a disease control rate (DCR) of 68% and 91%, and an objective response rate (ORR) of 12% and 17%, were achieved respectively, compared to 6% and 0% in the negative controls arm, with multi-protein drugs achieving significantly more durable responses. Thus, these approaches may effectively complement and expand current precision oncology approaches, as also illustrated by a case study.

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Section: Pharmacotypes Identification For Clinical Trial Designmentioning

confidence: 99%

Pre-clinical validation of an RNA-based precision oncology platform for patient-therapy alignment in a diverse set of human malignancies resistant to standard treatments

Mundi¹,

Cruz²,

Grunn³

et al. 2021

Preprint

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“…Treatment considerations for CNSET include wedge resections for smaller tumors and more substantial interventions in the form of partial hepatectomy or liver transplant for larger tumors. 22,28,29 Chemotherapy using soft tissue sarcoma or hepatoblastoma protocol has been reported in the literature. However, it is unclear whether chemotherapy has any significant role in preventing tumor recurrence, as the complete resection of tumor has proved curative in more than half of the reported cases.…”

Section: Treatment Considerationsmentioning

confidence: 99%

“…However, it is unclear whether chemotherapy has any significant role in preventing tumor recurrence, as the complete resection of tumor has proved curative in more than half of the reported cases. 29 Furthermore, preoperative chemotherapy has not shown effect on shrinkage or necrosis of the tumors in subsequent resections or imaging studies. 2,4,6,19,20,22,29 Thus, it is still uncertain whether chemotherapy holds any benefit with the current data available.…”

Section: Treatment Considerationsmentioning

confidence: 99%

Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review

Benedict

Zhang

2018

Archives of Pathology &Amp; Laboratory Medicine

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Calcifying nested stromal-epithelial tumor is a rare entity that has gone by a variety of names in the literature: ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor of the liver. To our knowledge, approximately 38 cases have been reported in the literature. The histogenesis is still largely unknown but histopathologically is characterized by nests of spindle and epithelioid cells in an organoid arrangement surrounded by a prominent dense myofibroblastic stroma with occasional psammomatous calcification and focal heterotopic ossification. Vascular invasion is rare and tumoral recurrence is uncommon with only a single reported case of metastasis leading to death. Treatment is mainly by surgical intervention with the role of chemotherapy seeming limited, but lack of data hinders a true recommendation. It is important to rule out other processes such as hepatoblastoma, calcified hemangioma, synovial sarcoma, metastatic gastrointestinal stromal tumor, desmoplastic small round cell tumor, among others, which appear similar radiographically and histopathologically.

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“…The tumor usually does not manifest early symptoms and is mostly diagnosed incidentally, as a singular mass averaging 12.6 cm in size ( 30 ). Laboratory results lack abnormalities, although some authors ( 2 , 4 , 7 , 11 , 12 , 21 , 25 , 28 ) reported the coexistence of CNSET with Cushing's Syndrome, therefore, elevated levels of adrenocorticotropic hormone (ACTH) may be observed.…”

Section: Introductionmentioning

confidence: 99%

Liver Transplantation for Unresectable Calcifying Nested Stromal Epithelial Tumor: Case Report With a 1-Year Follow-Up and Review of Literature

et al. 2022

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IntroductionCalcifying nested stromal epithelial tumor (CNSET) is an extremely rare diagnosis among patients treated for primary hepatic neoplasms. There are only 45 cases reported worldwide. Histopathological characteristics are well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma with variable calcification and ossification. It is mostly diagnosed in children and young females. Treatment strategies implemented for the management of CNSET include radiofrequency ablation, transarterial chemoembolization, surgical resection, adjuvant and neoadjuvant chemotherapy, and liver transplantation. Given the small number of available cases, there are still no established standards of treatment for this neoplasm.Case PresentationA 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient's clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment.ConclusionMultiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.

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Successful liver transplantation for non‐resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome

Cited by 12 publications

References 15 publications

Pre-clinical validation of an RNA-based precision oncology platform for patient-therapy alignment in a diverse set of human malignancies resistant to standard treatments

Pre-clinical validation of an RNA-based precision oncology platform for patient-therapy alignment in a diverse set of human malignancies resistant to standard treatments

Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review

Liver Transplantation for Unresectable Calcifying Nested Stromal Epithelial Tumor: Case Report With a 1-Year Follow-Up and Review of Literature

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