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Koh JS, Oh S, Chung C. Pulmonary lymphangioleiomyomatosis and renal angiomyolipoma in a patient with systemic lupus erythematosus: A case report. Medicine (Baltimore) 2022; 101:e30554. [PMID: 36197220 PMCID: PMC9509188 DOI: 10.1097/md.0000000000030554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
BACKGROUND The co-incidence of systemic lupus erythematosus (SLE) and tuberous sclerosis with pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML) is rare. In such patients, the rupture of renal AML may result in fatal circumstances, but this may be preventable. METHODS A 22-year-old Asian woman with SLE was admitted to our hospital with severe left-flank pain. Imaging studies showed the bilateral rupture of multiple renal AMLs. RESULTS The patient underwent emergency selective transcatheter embolization (TE) of the left renal artery. After TE and massive hydration, the patient complained of dyspnea and postembolization syndrome with fever. The chest computed tomography (CT) revealed pulmonary LAM, pulmonary edema with bilateral pleural effusions, and pneumonic consolidation. After the emergency procedure, the patient was treated with intravenous administration of antibiotics, diuretics, and nonsteroidal anti-inflammatory drugs for 10 days. The patient recovered favorably and was discharged 20 days after the treatment. She was diagnosed with renal AML and pulmonary LAM along with facial angiofibromas as well as tuberous sclerosis complex (TSC), although she had no TSC1 or TSC2 gene mutations. CONCLUSION Although rare, SLE may coexist with TSC, along with LAM and AML, with a risk of AML rupture. The activation of the mTOR signaling pathway is shared between SLE and TSC. Thus, in patients with SLE, clinicians should consider imaging studies, such as kidney sonography and chest CT, to screen for possible manifestation of AML and LAM.
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Affiliation(s)
- Jeong Suk Koh
- Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, College of Medicine, Chungnam National University, Daejeon, 301-721, Republic of Korea
| | - Sina Oh
- College of Medicine, Chungnam National University, Daejeon 35015, Republic of Korea
| | - Chaeuk Chung
- Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, College of Medicine, Chungnam National University, Daejeon, 301-721, Republic of Korea
- Infection Control Convergence Research Center, Chungnam National University School of Medicine, Daejeon 35015, Republic of Korea
- * Correspondence: Chaeuk Chung, Division of Pulmonology, Department of Internal Medicine, Chungnam National University, 282, Munhwaro, Jung-gu, Daejeon 35015, Republic of Korea (e-mail: )
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2
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John J, Aldera AP, Louw D, Lazarus J, Kesner K. Pulmonary lymphangioleiomyomatosis with an associated giant renal angiomyolipoma. Ther Adv Urol 2022; 14:17562872211069700. [PMID: 35096144 PMCID: PMC8796109 DOI: 10.1177/17562872211069700] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/08/2021] [Accepted: 12/10/2021] [Indexed: 11/25/2022] Open
Abstract
Pulmonary lymphangioleiomyomatosis (LAM), a rare, progressive disease predominantly affecting the lungs of women of reproductive age, is often associated with renal angiomyolipoma (AML). We report the case of a 29-year-old female patient who presented to our obstetrics department at 37 weeks’ gestation, complaining of abdominal pain, and constipation. Ultrasound noted a viable singleton with a large left-sided abdominal mass. After undergoing a caesarean section, she was referred to our urology department to assess her flank mass further. Computed tomography demonstrated a large, exophytic left renal mass measuring 22 cm x 16 cm x 13 cm, suggestive of an AML and numerous bilateral pulmonary cysts. A diagnosis of LAM and associated unilateral giant renal AML was made. As soon as she had fully recovered from her caesarean section, we removed the huge AML via a standard left-sided open nephrectomy without incident. We report this rare case of giant AML associated with LAM and review the literature about the association of these two conditions.
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Affiliation(s)
- Jeff John
- Division of Urology, Department of Surgery, Frere Hospital and Walter Sisulu University, East London 5200, South Africa
| | - Alessandro Pietro Aldera
- Division of Anatomical Pathology, University of Cape Town, Cape Town, South Africa; JDW Pathology Incorporated, Cape Town, South Africa
| | - Dap Louw
- Division of Urology, Department of Surgery, Frere Hospital and Walter Sisulu University, East London, South Africa
| | - John Lazarus
- Division of Urology, Department of Surgery, Groote Schuur Hospital and University of Cape Town, Cape Town, South Africa
| | - Ken Kesner
- Division of Urology, Department of Surgery, Frere Hospital and Walter Sisulu University, East London, South Africa
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3
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Chen HB, Xu XH, Yu CG, Wan MT, Feng CL, Zhao ZY, Mei DE, Chen JL. Tuberous sclerosis complex-lymphangioleiomyomatosis involving several visceral organs: A case report. World J Clin Cases 2021; 9:7085-7091. [PMID: 34540963 PMCID: PMC8409179 DOI: 10.12998/wjcc.v9.i24.7085] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2021] [Revised: 05/30/2021] [Accepted: 07/02/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease characterized by the proliferation, metastasis, and infiltration of smooth muscle cells in the lung and other tissues, which can be associated with tuberous sclerosis complex (TSC). The disorder of TSC has a variable expression, and there is great phenotypic variability.
CASE SUMMARY A 32-year-old Chinese woman with a history of multiple renal angioleiomyolipoma presented with a productive cough persisting for over 2 wk. High-resolution chest computed tomography revealed interstitial changes, multiple pulmonary bullae, bilateral pulmonary nodules, and multiple fat density areas of the inferior mediastinum. Conventional and contrast ultrasonography revealed multiple high echogenic masses of the liver, kidneys, retroperitoneum, and inferior mediastinum. These masses were diagnosed as angiomyolipomas. Pathology through thoracoscopic lung biopsy confirmed LAM. Furthermore, high-throughput genome sequencing of peripheral blood DNA confirmed the presence of a heterozygous mutation, c.1831C>T (p.Arg611Trp), of the TSC2 gene. The patient was diagnosed with TSC-LAM.
CONCLUSION We highlight a rare case of TSC-LAM and the first report of a mediastinum lymphangioleiomyoma associated with TSC-LAM.
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Affiliation(s)
- Hong-Bin Chen
- Department of Pulmonary and Critical Care Medicine, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Xiao-Hong Xu
- Department of Pulmonary and Critical Care Medicine, People's Hospital of Hanchuan, Hanchuan 431600, Hubei Province, China
| | - Cai-Gui Yu
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Meng-Ting Wan
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Chuang-Li Feng
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Zhi-Yu Zhao
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Dan-E Mei
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
| | - Jin-Ling Chen
- Department of Echocardiography, Renmin Hospital of Wuhan University, Wuhan 430060, Hubei Province, China
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4
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Chylous Ascites as a Presentation of Lymphangioleiomyomatosis. ACG Case Rep J 2021; 8:e00517. [PMID: 33681402 PMCID: PMC7932795 DOI: 10.14309/crj.0000000000000517] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/01/2020] [Accepted: 09/04/2020] [Indexed: 11/17/2022] Open
Abstract
A 35-year-old woman presented to the hospital with a 4-week history of large-volume chylous ascites refractory to paracentesis and new-onset dyspnea. Thoracic computed tomography revealed diffuse pulmonary cystic lesions with pleural effusions, and abdominal computed tomography showed ascites with large bilateral retroperitoneal masses displaying positron emission tomography avidity. Biopsy of the masses demonstrated lymphatic invasion by a perivascular epithelioid cell neoplasm, a smooth muscle tumor. The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement.
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5
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Clements D, Miller S, Johnson SR. Pulmonary Lymphangioleiomyomatosis originates in the pleural mesothelial cell population. Med Hypotheses 2020; 141:109703. [PMID: 32276237 DOI: 10.1016/j.mehy.2020.109703] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Accepted: 03/30/2020] [Indexed: 02/07/2023]
Abstract
Lymphangioleiomyomatosis (LAM) is a cystic lung disease mainly affecting women, in which degradation of the lung parenchyma is associated with a cell of unknown provenance, known as a LAM cell. LAM cells carry TSC2 mutations and can be identified in the lung parenchyma by their expression of both smooth muscle actin and antigens characteristic of melanocytes and melanocytic tumors. The nature of the cell-of-origin of LAM is controversial, and despite continued research effort remains elusive. Further, it has not been possible to culture pulmonary LAM cells in vitro, and current research relies on cells and animal models which may not recapitulate all features of the disease. We noted aberrant expression of melanoma antigens in pleural mesothelial cells in lung tissue from LAM patients, indicating that these cells could be the precursors of parenchymal LAM cells. We hypothesise that loss of tuberin function following TSC2 mutation in the mesothelial cell lineage gives rise to the cell-of-origin of pulmonary LAM (P-LAM), and of other associated conditions commonly noted in LAM patients. The unique properties of mesothelial cells provide a straightforward explanation of the diverse presentation of LAM.
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Affiliation(s)
- D Clements
- Division of Respiratory Medicine, University of Nottingham Biodiscovery Institute, University Park, Nottingham NG7 2RD, UK.
| | - S Miller
- Division of Respiratory Medicine, University of Nottingham Biodiscovery Institute, University Park, Nottingham NG7 2RD, UK
| | - S R Johnson
- Division of Respiratory Medicine, University of Nottingham Biodiscovery Institute, University Park, Nottingham NG7 2RD, UK; National Centre for Lymphangioleiomyomatosis, Nottingham University Hospitals NHS Trust, Queen's Medical Centre, Nottingham NG7 2UH, UK
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6
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Tsili AC, Ntorkou A, Argyropoulou MI. Renal Epithelioid Angiomyolipoma Associated with Pulmonary Lymphangioleiomyomatosis: Imaging Findings. J Clin Imaging Sci 2017; 7:18. [PMID: 28584685 PMCID: PMC5450460 DOI: 10.4103/jcis.jcis_14_17] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2017] [Accepted: 03/27/2017] [Indexed: 12/16/2022] Open
Abstract
Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. There are few reports in the English literature addressing on the imaging findings of renal EAMLs, which are considered nonspecific. We present the sonographic, computed tomographic, and magnetic resonance imaging findings of a renal EAML in a pregnant woman with concomitant pulmonary lesions indicative of LAM, without stigmata of TS. We conclude the importance of considering EAML as a possible diagnosis in the presence of a large renal mass with high cellular content and small amounts of fat in the coexistence of pulmonary LAM.
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Affiliation(s)
- Athina C Tsili
- Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina, Greece
| | - Alexandra Ntorkou
- Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina, Greece
| | - Maria I Argyropoulou
- Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina, Greece
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7
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Abstract
Lymphangioleiomyomatosis (LAM) is a devastating rare lung disease affecting primarily childbearing age women in which tumors consisting of abnormal smooth-muscle-like cells grow within the lungs and progressively lead to loss of pulmonary function. LAM cells metastasize to the lungs, predominantly through the lymphatics; however, the source of the LAM cell is still unknown. LAM cells contain inactivating mutations in genes encoding tuberous sclerosis 1 or 2, proteins that normally limit cell growth through suppression of mammalian target of rapamycin complex 1. As of today, sirolimus (an mammalian target of rapamycin complex 1 inhibitor) is the only treatment, available for LAM patients that is approved by the Food and Drug Administration; however, this drug and others in its class provide stabilization but not remission of LAM. One of the biggest problems in treating LAM is that both the origin of the LAM cells and the mechanism of the sexual dimorphism in LAM are still not understood. LAM cells express estrogen and progesterone receptors, and lung function declines during periods of high circulating estrogen levels. Moreover, numerous basic research studies find that estrogen is a key driving force in LAM cell proliferation, migration, and metastasis. In this review, we highlight recent insights regarding the role of steroid hormones in LAM and discuss possible explanations for the profound female sexual dimorphism of LAM.
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Affiliation(s)
- Hen Prizant
- Departments of Medicine and Pharmacology, University of Rochester Medical Center, Rochester, New York 14642
| | - Stephen R Hammes
- Departments of Medicine and Pharmacology, University of Rochester Medical Center, Rochester, New York 14642
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8
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Lymphangioleiomyomatosis Presenting with Perirenal Hemorrhage. J Belg Soc Radiol 2015; 99:46-49. [PMID: 30039066 PMCID: PMC6032798 DOI: 10.5334/jbr-btr.836] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
Lymphangioleiomyomatosis (LAM) is a rare debilitating disease of unknown etiology, classically described as almost exclusively affecting women of childbearing age. The disease most commonly involves the lungs and is characterized by hamartomatous smooth muscle cell proliferations along blood vessels, airways and lymphatics. Most patients present with pulmonary symptoms, including shortness of breath, recurrent pneumothorax and pleural effusions. Extrapulmonary manifestations of LAM as the initial presentation of the disease are highly unusual. We present the case of a patient in whom LAM was incidentally discovered when the patient presented with retroperitoneal hemorrhage from a ruptured renal angiomyolipoma.
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9
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Familial syndromes associated with intracranial tumours: a review. Childs Nerv Syst 2014; 30:47-64. [PMID: 24193148 DOI: 10.1007/s00381-013-2309-z] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2013] [Accepted: 10/15/2013] [Indexed: 12/15/2022]
Abstract
BACKGROUND Most cancers of the central nervous system (CNS) occur sporadically in the absence of any known underlying familial disorder or multi-systemic syndrome. Several syndromes are associated with CNS malignancies, however, and their recognition has significant implications for patient management and prognosis. Patients with syndrome-associated CNS malignancies often have multiple tumours (either confined to one region or distributed throughout the body), with similar or different histology. OBJECTIVE This review examines syndromes that are strongly associated with CNS cancers: the phakomatosis syndromes, familial syndromes such as Li-Fraumeni and familial polyposis syndromes and dyschondroplasia.
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10
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Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis. Proc Natl Acad Sci U S A 2013; 110:14960-5. [PMID: 23983265 DOI: 10.1073/pnas.1309110110] [Citation(s) in RCA: 55] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women associated with the metastasis of tuberin-null cells with hyperactive mammalian target of rapamycin complex 1 (mTORC1) activity. Clinical trials with the mTORC1 inhibitor rapamycin have revealed partial efficacy but are not curative. Pregnancy appears to exacerbate LAM, suggesting that estrogen (E2) may play a role in the unique features of LAM. Using a LAM patient-derived cell line (bearing biallelic Tuberin inactivation), we demonstrate that E2 stimulates a robust and biphasic activation of ERK2 and transcription of the late response-gene Fra1 associated with epithelial-to-mesenchymal transition. In a carefully orchestrated collaboration, activated mTORC1/S6K1 signaling enhances the efficiency of Fra1 translation of Fra1 mRNA transcribed by the E2-ERK2 pathway, through the phosphorylation of the S6K1-dependent eukaryotic translation initiation factor 4B. Our results indicate that targeting the E2-ERK pathway in combination with the mTORC1 pathway may be an effective combination therapy for LAM.
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11
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Wu PH, Hwang DY, Kuo MC, Hwang SJ, Chen HC. Pneumothorax in a female with renal angiomyolipoma. Kaohsiung J Med Sci 2011; 27:469-72. [PMID: 21943821 DOI: 10.1016/j.kjms.2011.06.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2010] [Accepted: 10/21/2010] [Indexed: 11/25/2022] Open
Abstract
Spontaneous pneumothorax occurs less in females than in males, in contrast to the relatively more commonly and incidentally found renal angiomyolipoma (AML). We report a renal AML in a 23-year-old female patient, which presented as right palpable abdominal mass. This renal tumor was removed owing to enlargement and internal hemorrhage and AML was proved by pathological examination. Three years later, she experienced a life-threatening spontaneous tension pneumothorax and pulmonary lymphangioleiomyomatosis (LAM), which was diagnosed by chest computed tomography. Clinically, she did not have tuberous sclerosis. Pulmonary LAM and renal AML are related lesions and both of these proliferative lesions occur in sporadic patients without family history and at much higher frequency in patients with tuberous sclerosis. Because of the nonspecific symptoms and high rate of complications, we should consider the possibility of LAM in patients diagnosed with AML. The association between renal AML and pulmonary LAM is reviewed.
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Affiliation(s)
- Ping-Hsun Wu
- Department of Internal Medicine, Kaohsiung Medical University Hospital, Taiwan
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12
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Er A, Yıldırım CA. Giant renal angiomyolipomatosis in association with pulmonary lymphangiomyomatosis. Intern Med J 2010; 40:384-5. [DOI: 10.1111/j.1445-5994.2010.02218.x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
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13
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Barnes EA, Kenerson HL, Mak BC, Yeung RS. The loss of tuberin promotes cell invasion through the ß-catenin pathway. Am J Respir Cell Mol Biol 2009; 43:617-27. [PMID: 20042714 DOI: 10.1165/rcmb.2008-0335oc] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
Mutations in the tumor suppressor tuberin (TSC2) are a common factor in the development of lymphangioleiomyomatosis (LAM). LAM is a cystic lung disease that is characterized by the infiltration of smooth muscle-like cells into the pulmonary parenchyma. The mechanism by which the loss of tuberin promotes the development of LAM has yet to be elucidated, although several lines of evidence suggest it is due to the metastasis of tuberin-deficient cells. Here we show that tuberin-null cells become nonadherent and invasive. These nonadherent cells express cleaved forms of β-catenin. In reporter assays, the β-catenin products are transcriptionally active and promote MMP7 expression. Invasion by the tuberin-null cells is mediated by MMP7. Examination of LAM tissues shows the expression of cleaved β-catenin products and MMP7 consistent with a model that tuberin-deficient cells acquire invasive properties through a β-catenin-dependent mechanism, which may underlie the development of LAM.
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Affiliation(s)
- Elizabeth A Barnes
- University of Washington, Department of Surgery, Seattle, Washington 98195, USA
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14
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A right renal angiomyolipoma with IVC thrombus and pulmonary embolism. Int Urol Nephrol 2009; 42:305-8. [DOI: 10.1007/s11255-009-9612-3] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2009] [Accepted: 06/22/2009] [Indexed: 10/20/2022]
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15
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Higa F, Uchihara T, Haranaga S, Yara S, Tateyama M, Oshiro Y, Shiraishi M, Kumasaka T, Seyama K, Fujita J. Malignant epithelioid angiomyolipoma in the kidney and liver of a patient with pulmonary lymphangioleiomyomatosis: lack of response to sirolimus. Intern Med 2009; 48:1821-5. [PMID: 19834275 DOI: 10.2169/internalmedicine.48.2411] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 26-year-old woman with lymphangioleiomyomatosis (LAM) was hospitalized for the surgical excision of a giant abdominal tumor of right kidney origin. The pathological diagnosis of the tumor was conventional angiomyolipoma (AML). After 8 months, 2 liver tumors appeared and grew rapidly. The tumors were resected, and the pathological finding of these tumors was epithelioid AML. Thereafter, metastatic multiple lung tumors appeared, and there was local recurrence of the liver tumors. Sirolimus, an mTOR protein inhibitor, was used to treat epithelioid AML. However, the drug did not inhibit the rapid growth of the tumor at all. This finding suggests that sirolimus might not be effective against epithelioid AML, and in such cases, complete surgical resection should be the treatment of choice.
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Affiliation(s)
- Futoshi Higa
- Department of Medicine and Therapeutics, Prevention, Control of Infectious Diseases, School of Medicine, University of the Ryukyus, Okinawa.
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Regression of Pulmonary Lymphangioleiomyomatosis (PLAM)-Associated Retroperitoneal Angiomyolipoma Post–Lung Transplantation With Rapamycin Treatment. J Heart Lung Transplant 2008; 27:1268. [DOI: 10.1016/j.healun.2008.08.002] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2008] [Revised: 07/25/2008] [Accepted: 08/06/2008] [Indexed: 11/19/2022] Open
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17
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Sam R, Khalid S, Brecklin C, Schwartz M, Dunea G. A case of lymphangioleiomyomatosis with membranous nephropathy and likely systemic lupus. Clin Exp Nephrol 2008; 13:166-169. [PMID: 18820829 DOI: 10.1007/s10157-008-0083-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2008] [Accepted: 08/05/2008] [Indexed: 10/21/2022]
Abstract
Membranous glomerulonephritis is most often idiopathic, but it can be secondary to systemic lupus erythematosus, viral hepatitis, and drugs. A number of malignancies have also been associated with membranous glomerulonephritis, although a causal link has not been established yet. A young patient with lymphangioleiomyomatosis is described who developed massive proteinuria and was found to have membranous glomerulonephritis on renal biopsy. There were many pathological features and some clinical features that suggested the patient also suffered from systemic lupus erythematosus.
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Affiliation(s)
- Ramin Sam
- Division of Nephrology, San Francisco General Hospital, 1001 Potrero Ave, Building 100, Rm 349, San Francisco, CA, 94110-1341, USA. .,Division of Nephrology, The University of California, San Francisco, CA, USA.
| | - Samra Khalid
- Department of Medicine, Stroger Hospital of Cook County, Chicago, IL, 60612, USA
| | - Carolyn Brecklin
- Division of Nephrology, Stroger Hospital of Cook County, Chicago, IL, 60612, USA.,Section of Nephrology, University of Illinois at Chicago, Chicago, IL, 60612, USA
| | - Melvin Schwartz
- Division of Pathology, Rush Medical College, Chicago, IL, 60612, USA
| | - George Dunea
- Division of Nephrology, Stroger Hospital of Cook County, Chicago, IL, 60612, USA.,Hektoen Institute of Medicine, Chicago, IL, 60612, USA
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18
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Kim JW, Lee TW, Kim MJ, Oh MM, Bae JH, Park HS, Kim JJ, Shim KS. Spontaneous Rupture of Renal Angiomyolipoma in a Female Tuberous Sclerosis Patient with Pulmonary Lymphangioleiomyomatosis. Korean J Urol 2007. [DOI: 10.4111/kju.2007.48.3.344] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
Affiliation(s)
- Jong Wook Kim
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Tae Won Lee
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Myung Joon Kim
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Mi Mi Oh
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Jae Hyun Bae
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Hong Seok Park
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Je Jong Kim
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
| | - Kang Soo Shim
- Department of Urology, College of Medicine, Korea University, Seoul, Korea
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19
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Ponce Díaz-Reixa J, Barbagelata López A, Romero Selas E, Marcos Rodríguez P, Sánchez Rodríguez-Losada J, Alvarez Castelo L, Fernández Rosado E, González Martín M. [Renal angiomyolipomatosis and pulmonary lymphangiomyomatosis. Its relationship with Bourneville syndrome]. Actas Urol Esp 2006; 30:386-93. [PMID: 16838610 DOI: 10.1016/s0210-4806(06)73462-3] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
INTRODUCTION Renal angiomyolipomas are not very frequent in urologic activities. Pulmonary lymphangioleiomyomatosis is a rare disease that occurs mainly in women of reproductive age. Tuberous sclerosis shows lesions to different organs, including these both entities. MATERIAL AND METHOD We retrospectively analyze patients treated in our center. Three most representatives cases are reported. An indexed literature review is done, in order to establish a clinical protocol to manage these kinds of patients. RESULTS [corrected] Between 1990 and 2004, eight patients have been attended with an angiomyolipoma renal diagnostic. Five women (62%) and three men (38%). Medium age is 52,3y (29-69). In 3 of them (all women), it was also done a diagnostic of pulmonary lymphangioleiomyomatosis. CONCLUSIONS Bourneville syndrome is not very frequent. Associated angiomyolipomas usually are multiple, bilateral, with tender to grow and require more interventions. Previous diagnostic let us prevent future complications with vigilance, selective arterial embolization and nephron-spare surgery. Tumour size and the presence of symptoms are more decisive to decide best therapy alternative. Young patients with pulmonary lymphangioleiomyomatosis should be advise against pregnancy and the use of preparations containing estrogens.
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