Pure epithelial neuroendocrine neoplasms of the bladder: clinicopathological characteristics of 2 cases and literature review

doi:10.16150/j.1671-2870.2024.06.007

Abstract

Abstract:

Objective To investigate the clinicopathological features and prognosis of pure epithelial neuroendocrine neoplasms (NEN) of the bladder. Methods A retrospective analysis was conducted on the clinical data, histopathological morphology, and immunohistochemistry of 2 patients with pure epithelial NEN of the bladder admitted between 2018 and 2024. A differential diagnosis was performed, and a detailed literature review was conducted. Results Both patients were females, aged 85 (Case 1) and 84 (Case 2), presenting with lower limb edema and hematuria, respectively. B-scan ultrasound and MRI examinations showed a polypoid hypoechoic lesion in the trigone of the bladder in Case 1, with no enhancement on MRI, suggesting a benign lesion. Case 2 showed a nodular hypoechoic lesion in the anterior bladder wall with significant enhancement on MRI, suggesting a malignant tumor, with paraganglioma not excluded. Gross examination revealed that the two cases had one polypoid tissue and one nodular tissue, respectively, with maximum diameters ranging from 0.6 cm to 3.0 cm. Case 1 had a smooth surface, while Case 2 had a grayish-yellow cross section with medium hardness. Microscopically, both tumors showed typical NEN histopathological features, with the surface covered by bladder mucosa. Case 1 was confined to the lamina propria, while Case 2 showed invasive growth into the muscularis propria. Case 1 was diagnosed as well-differentiated neuroendocrine tumor (WD-NET) and Case 2 as large cell neuroendocrine carcinoma (LCNEC). Immunohistochemically, both tumor cells diffusely expressed broad-spectrum cytokeratin and neuroendocrine markers (2/2), with wild-type expression of P53 (2/2). The Ki67 proliferation index was 3%-5% in Case 1, with focal PSAP expression, and 60%-70% in Case 2, with minimal GATA-3 expression. Neither patient received special treatment. Case 1 was followed up for 76 months with no recurrence, while Case 2 died of recurrence and lung metastasis 3 months after surgery. Literature review suggested that pure epithelial NEN of the bladder exhibited typical histopathological morphology and immunophenotype of NEN. WD-NET typically followed an indolent course, while LCNEC was highly invasive and prone to recurrence and metastasis, consistent with the two cases reported in this study. Conclusions Pure epithelial NEN of the bladder is extremely rare. The definitive diagnosis mainly relies on histopathological examination and immunohistochemical staining. WD-NET has a good prognosis after complete tumor resection while LCNEC is highly invasive and has a poor prognosis.

Key words: Bladder, Pure neuroendocrine neoplasm, Well-differentiated neuroendocrine tumors, Large cell neuroendocrine carcinoma

CLC Number:

R737.14
R445

ZHOU Henghua, LIN Lan, ZHU Guixiang, LIU Min, HUANG Wentao. Pure epithelial neuroendocrine neoplasms of the bladder: clinicopathological characteristics of 2 cases and literature review[J]. Journal of Diagnostics Concepts & Practice, 2024, 23(06): 602-611.

Figures/Tables 10

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Table 1

Table 2

Clinicopathological features and prognosis of 32 cases of bladder LCNEC

Reference	Number	Age	Gender	Site	Size (cm)	Clinical feature	Staging	Treatment	Follow up and prognosis (mo)
[5]	1	73	M	Posterior wall	4.0	Hematuria	pT_3b	RC+LND	2,died of RE/ME
[23]	1	32	M	Anterior superior wall	3.0	Hematuria	pT₃	PC+CT	12,alive with RE/ME
[24]	2	40/43	M/F=1:1	NA	NA	NA	pT₂，pT₄	RC+CT，RC+RT	13,NED；12,died of ME
[25]	1	37	M	Posterior wall	2.5	Hematuria	pT_3b	RC+LND+CT	22,NED
[26]	1	19	M	NA	NA	NA	NA	PC+CT	14,died of ME
[27]	1	74	M	Left wall	NA	Brain metastases	NA	PC+CT+RT	5,died of pulmonary embolism
[28]	1	67	M	Left posterior lateral wall	5.0	Hematuria	pT₂	TURBT	0.5,died of heart failure
[29]	1	59	M	Bottom	Huge	Dysuria and hematuria	pT_4a	TURBT	NA
[30]	1	68	M	Top wall	2.8	Hematuria	pT_2b	RC+LND+CT	30,died of RE/ME
[31]	1	84	M	Posterior wall	NA	Hematuria	NA	TURBT+CT	NA
[32]	1	68	M	Top wall	2.8	Hematuria	pT_2b	RC+LND	NA
[33]	1	70	M	Left wall	3.5	Hematuria	pT_2b	TURBT	7,died of ME
[34]	1	58	M	Left and anterior walls	6.5	Hematuria	pT_3b	RC	5,died of ME
[35]	5	Mean71.8	M/F=4/1	NA	NA	NA	3,pT_3a/2,pT_4a	RC(5/5);1 NACT	2.4-116.4(Mean14.4), 3 died of RE/2 died from other
[36]	1	72	M	Bottom	NA	Back pain and acute renal failure	pT_4a	RC+CT	36,RE with regression
[37]	1	72	M	Diffuse	NA	Hematuria	pT₂	RC+CT+IT	11,died of ME
[38]	1	45	M	Left wall	4.0	Acute renal failure	NA	TURBT+CT+RT	NA
[39]	1	39	M	Right wall	4.1	Hematuria	pT_2b	RC+LND+CT	59,NED
[40]	1	30	M	Anterior wall	3.4	Hematuria	pT_3b	PC+CT	24,NED
[41]	1	49	M	Left wall	7.0	Vertebral metastasis	pT₂	TURBT+CT+RT	12,Pain improvemen
[42]	1	66	M	Left posterior wall	4.3	Hematuria	pT_3b	NA	10,NED
[43]	1	67	M	Right posterior wall and right ureteral orifice	4.4	Hematuria	pT_3a	RC+LND+CT	39,NED
[44]	1	56	F	Anterior wall, posterior wall, and trigone	3.8	Hematuria	pT₂	RC+CT	6,Lost follow-up
[45]	1	79	M	Left wall	5.0	Hematuria	pT₃	RC+IT	24,RE
[46]	1	72	M	Anterior wall	2.0	Hematuria	pT_2b	PC	10,NED
[47]	1	65	M	Right anterior wall	3.0	Hematuria	pT_2a	PC+CT	7,NED
Present case	1	84	F	Anterior wall	3.0	Urinary frequency, urgency, and hematuria	pT_3a	PC	3, died of RE/ME

Table 2

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