Juxtaglomerular cell tumor

Last updated
Juxtaglomerular cell tumor
Other namesReninoma
Specialty Nephrology/oncology

Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells, with fewer than 100 cases reported in literature. This tumor typically secretes renin, hence the former name of reninoma. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain. [1]

Contents

Pathophysiology

By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, [2] and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. [3] Both of these conditions may be corrected by surgical removal of the tumor. [4] Asymptomatic cases have been reported. [5]

Histopathology

JCT is morphologically characterized by multiple foci malignant mesenchymal epithelioid cells with, often with admixed necrosis, and a perivascular growth pattern. The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin. [6]

Diagnosis

Clinically, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT. Other kidney tumors can cause hypertension by secreting renin. JCTs have a variable appearance and have often being misdiagnosed as renal cell carcinomas; dynamic computed tomography is helpful in the differential diagnosis. [7]

Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms. [6] [8]

Prognosis

JCT is often described as benign and one case of metastasis has been reported, so its malignant potential is uncertain. [1] In most cases the tumor is encapsulated. [9]

History

Juxtaglomerular cell tumor was first described in 1967 in a paper by Robertson et al., and first named by Kihara et al. in 1968. Since then, approximately 100 case reports have been published. [5] Karyotyping of a small number of these tumors revealed a common loss of chromosomes 9 and 11. [1]

Related Research Articles

<span class="mw-page-title-main">Renin</span> Aspartic protease protein and enzyme

Renin, also known as an angiotensinogenase, is an aspartic protease protein and enzyme secreted by the kidneys that participates in the body's renin–angiotensin–aldosterone system (RAAS)—also known as the renin–angiotensin–aldosterone axis—that increases the volume of extracellular fluid and causes arterial vasoconstriction. Thus, it increases the body's mean arterial blood pressure.

<span class="mw-page-title-main">Renin–angiotensin system</span> Hormone system

The renin–angiotensin system (RAS), or renin–angiotensin–aldosterone system (RAAS), is a hormone system that regulates blood pressure, fluid and electrolyte balance, and systemic vascular resistance.

<span class="mw-page-title-main">Kidney cancer</span> Medical condition

Kidney cancer, also known as renal cancer, is a group of cancers that starts in the kidney. Symptoms may include blood in the urine, lump in the abdomen, or back pain. Fever, weight loss, and tiredness may also occur. Complications can include spread to the lungs or brain.

<span class="mw-page-title-main">Primary aldosteronism</span> Medical condition

Primary aldosteronism (PA), also known as primary hyperaldosteronism or Conn's syndrome, refers to the excess production of the hormone aldosterone from the adrenal glands, resulting in low renin levels and high blood pressure. This abnormality is caused by hyperplasia or tumors. Many experience fatigue, potassium deficiency and high blood pressure which may cause poor vision, confusion or headaches. Symptoms may also include: muscular aches and weakness, muscle spasms, low back and flank pain from the kidneys, trembling, tingling sensations, dizziness/vertigo, nocturia and excessive urination. Complications include cardiovascular disease such as stroke, myocardial infarction, kidney failure and abnormal heart rhythms.

<span class="mw-page-title-main">Juxtaglomerular apparatus</span> Structure that regulates function of each nephron

The juxtaglomerular apparatus is a structure in the kidney that regulates the function of each nephron, the functional units of the kidney. The juxtaglomerular apparatus is named because it is next to (juxta-) the glomerulus.

<span class="mw-page-title-main">Renal artery stenosis</span> Medical condition

Renal artery stenosis (RAS) is the narrowing of one or both of the renal arteries, most often caused by atherosclerosis or fibromuscular dysplasia. This narrowing of the renal artery can impede blood flow to the target kidney, resulting in renovascular hypertension – a secondary type of high blood pressure. Possible complications of renal artery stenosis are chronic kidney disease and coronary artery disease.

<span class="mw-page-title-main">Fibrosarcoma</span> Medical condition

Fibrosarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. Fibrosarcomas mainly arise in people between the ages of 25 and 79. It originates in fibrous tissues of the bone and invades long or flat bones such as the femur, tibia, and mandible. It also involves the periosteum and overlying muscle.

<span class="mw-page-title-main">Juxtaglomerular cell</span> Cell in kidneys that produces & secretes renin

Juxtaglomerular cells, also known as juxtaglomerular granular cells are cells in the kidney that synthesize, store, and secrete the enzyme renin. They are specialized smooth muscle cells mainly in the walls of the afferent arterioles that deliver blood to the glomerulus. In synthesizing renin, they play a critical role in the renin–angiotensin system and thus in autoregulation of the kidney.

Secondary hypertension is a type of hypertension which by definition is caused by an identifiable underlying primary cause. It is much less common than the other type, called essential hypertension, affecting only 5-10% of hypertensive patients. It has many different causes including endocrine diseases, kidney diseases, and tumors. It also can be a side effect of many medications.

<span class="mw-page-title-main">Arteriolosclerosis</span> Medical condition

Arteriolosclerosis is a form of cardiovascular disease involving hardening and loss of elasticity of arterioles or small arteries and is most often associated with hypertension and diabetes mellitus. Types include hyaline arteriolosclerosis and hyperplastic arteriolosclerosis, both involved with vessel wall thickening and luminal narrowing that may cause downstream ischemic injury. The following two terms whilst similar, are distinct in both spelling and meaning and may easily be confused with arteriolosclerosis.

<span class="mw-page-title-main">Hyperaldosteronism</span> Hormonal disorder

Hyperaldosteronism is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia) and increased hydrogen ion excretion (alkalosis).

Malignant rhabdoid tumour (MRT) is a very aggressive form of tumour originally described as a variant of Wilms' tumour, which is primarily a kidney tumour that occurs mainly in children.

<span class="mw-page-title-main">Neprilysin</span> Mammalian protein found in Homo sapiens

Neprilysin, also known as membrane metallo-endopeptidase (MME), neutral endopeptidase (NEP), cluster of differentiation 10 (CD10), and common acute lymphoblastic leukemia antigen (CALLA) is an enzyme that in humans is encoded by the MME gene. Neprilysin is a zinc-dependent metalloprotease that cleaves peptides at the amino side of hydrophobic residues and inactivates several peptide hormones including glucagon, enkephalins, substance P, neurotensin, oxytocin, and bradykinin. It also degrades the amyloid beta peptide whose abnormal folding and aggregation in neural tissue has been implicated as a cause of Alzheimer's disease. Synthesized as a membrane-bound protein, the neprilysin ectodomain is released into the extracellular domain after it has been transported from the Golgi apparatus to the cell surface.

<span class="mw-page-title-main">Mesoblastic nephroma</span> Medical condition

Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first three months of life and accounts for 3-5% of all childhood renal neoplasms. This neoplasm is generally non-aggressive and amenable to surgical removal. However, a readily identifiable subset of these kidney tumors has a more malignant potential and is capable of causing life-threatening metastases. Congenital mesoblastic nephroma was first named as such in 1967 but was recognized decades before this as fetal renal hamartoma or leiomyomatous renal hamartoma.

<span class="mw-page-title-main">Perivascular epithelioid cell tumour</span> Medical condition

Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). These are rare tumours that can occur in any part of the human body.

<span class="mw-page-title-main">Renal oncocytoma</span> Medical condition

A renal oncocytoma is a tumour of the kidney made up of oncocytes, epithelial cells with an excess amount of mitochondria.

<span class="mw-page-title-main">Clear cell sarcoma</span> Rare form of cancer

Clear cell sarcoma is a rare form of cancer called a sarcoma. It is known to occur mainly in the soft tissues and dermis. Rare forms were thought to occur in the gastrointestinal tract before they were discovered to be different and redesignated as gastrointestinal neuroectodermal tumors.

<span class="mw-page-title-main">Metanephric adenoma</span> Medical condition

Metanephric adenoma (MA) is a rare, benign tumour of the kidney, that can have a microscopic appearance similar to a nephroblastoma, or a papillary renal cell carcinoma.

Page kidney or Page phenomena is a potentially reversible form of secondary arterial hypertension caused by external compression of the renal parenchyma by some perirenal process. Any process that causes mass effect can be a potential cause of Page kidney. Hematomas, urinomas, tumors, cysts, lymphoceles, and aneurysms have all been reported in the literature. The compression is believed to cause activation of the renin–angiotensin–aldosterone system (RAAS) via microvascular ischemia.

Sclerosing epithelioid fibrosarcoma (SEF) is a very rare malignant tumor of soft tissues that on microscopic examination consists of small round or ovoid neoplastic epithelioid fibroblast-like cells, i.e. cells that have features resembling both epithelioid cells and fibroblasts. In 2020, the World Health Organization classified SEF as a distinct tumor type in the category of malignant fibroblastic and myofibroblastic tumors. However, current studies have reported that low-grade fibromyxoid sarcoma (LGFMS) has many clinically and pathologically important features characteristic of SEF; these studies suggest that LGSFMS may be an early form of, and over time progress to become, a SEF. Since the World Health Organization has classified LGFMS as one of the malignant fibroblastic and myofibroblastic tumors that is distinctly different than SEF, SEF and LGFMS are here regarded as different tumor forms.

References

  1. 1 2 3 Capovilla M, Couturier J, Molinié V, Amsellem-Ouazana D, Priollet P, Baumert H, Bruneval P, Vieillefond A (March 2008). "Loss of chromosomes 9 and 11 may be recurrent chromosome imbalances in juxtaglomerular cell tumors". Hum. Pathol. 39 (3): 459–62. doi:10.1016/j.humpath.2007.08.010. PMID   18261631.
  2. W. Hanna; et al. (April 2, 1979). "Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension". Can Med Assoc J. 120 (8): 957–9. PMC   1819229 . PMID   436071.
  3. Beaudoin, J.; Périgny M; Têtu B; Lebel M. (2008). "A Patient With A Juxtaglomerular Cell Tumor With Histological Vascular Invasion". Nature Clinical Practice Nephrology. 4 (8): 458–62. doi:10.1038/ncpneph0890. PMID   18654602. S2CID   21445618.
  4. Wong L, Hsu TH, Perlroth MG, Hofmann LV, Haynes CM, Katznelson L (February 2008). "Reninoma: case report and literature review". J. Hypertens. 26 (2): 368–73. doi:10.1097/HJH.0b013e3282f283f3. PMID   18192852. S2CID   44701879.
  5. 1 2 Kuroda, Naoto; et al. (2011). "Review of juxtaglomerular cell tumor with focus on pathobiological aspect". Diagnostic Pathology. 6: 80. doi: 10.1186/1746-1596-6-80 . PMC   3173291 . PMID   21871063.
  6. 1 2 Cucchiari D, Bertuzzi A, Colombo P, De Sanctis R, Faucher E, Fusco N, Pellegrinelli A, Arosio P, Angelini C (May 2013). "Juxtaglomerular cell tumor: multicentric synchronous disease associated with paraneoplastic syndrome". J Clin Oncol. 31 (14): e240–2. doi: 10.1200/JCO.2012.43.5545 . PMID   23547072.
  7. Tanabe; et al. (July 2001). "Dynamic computer tomography is useful in the differential diagnosis of juxtaglomerular cell tumor and renal cell carcinoma.Tanab". Hypertens. Res. 24 (4): 331–6. doi: 10.1291/hypres.24.331 . PMID   11510743.
  8. Martin, S. A.; Mynderse, L. A.; Lager, D. J.; Cheville, J. C.; Martin; Lager; Cheville (December 2001). "Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature". American Journal of Clinical Pathology . 116 (6): 854–63. doi: 10.1309/B10J-FKQ5-J7P8-WKU4 . PMID   11764074.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  9. Abbi RK, McVicar M, Teichberg S, Fish L, Kahn E (1993). "Pathologic characterization of a renin-secreting juxtaglomerular cell tumor in a child and review of the pediatric literature". Pediatr. Pathol. 13 (4): 443–51. doi:10.3109/15513819309048234. PMID   8372029.