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Retroperitoneal Lymphoma

Primary Retroperitoneal Lymphoma

Primary retroperitoneal lymphomas are a rare occurrence in clinical practice and their non-specific clinical presentation comprised primarily of constitutional symptoms. The diagnosis is difficult to attain without an extensive clinical and radiological workup, whereas a biopsy is necessary to confirm hematologic malignancy and exclude other possible etiologies.


Presentation

Although lymphomas are considered to be one of the most common malignancies of the retroperitoneum, this location has been very rarely reported as the primary site [1] [2] [3]. Based on the underlying subtype, manifestations of the disease usually appear in different age groups. Namely, non-Hodgkin lymphomas are most frequently encountered in individuals between 40-70 years of age, whereas Hodgkin lymphoma has two peaks - at the end of adolescence and in late adulthood [1]. It is not uncommon for lymphomas to affect various organs (liver, spleen, the gastrointestinal tract) and multiple lymph nodes, but despite its frequent systemic involvement, the clinical presentation commonly starts with a non-specific swelling of the abdomen and discomfort [4]. Additional signs include painless lymphadenopathy (most commonly of the cervical lymph nodes), fever, weight loss, night sweats, and a palpable mass in the abdomen often discovered incidentally, which is more commonly identified in patients suffering from a Hodgkin lymphoma [4] [5]. Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic [5] [6].

Hodgkin's Disease
  • disease with enlarged retroperitoneal and mesenteric lymphe nodes Retroperitoneal lymphe nodes: Malignant lymphoma with a hypoechoic vascularized mass around the aorta Retroperitoneal lymphe nodes: Mass around the aorta that biopsy proved to be a NHL[ultrasoundcases.info]
  • .: The accuracy of retroperitoneal ultrasonography in Hodgkin’s disease and non Hodgkin’s lymphoma. Radiology 125:485–487, 1977. PubMed Google Scholar Forsythe, J. R., Gosink, B. B. and Leopold, G.[link.springer.com]
  • Peripancreatic lymph node enlargement in Hodgkin's disease, non-Hodgkin's lymphoma, and pancreatic carcinoma. J Comput Tomogr 1984; 8:1-11. [More details] Earl HM, Sutcliffe SB, Fry IK, Tucker AK, Young J, Husband J, et al.[joplink.net]
  • Epidemiology NHL is more than five times as common as Hodgkin's disease.[patient.info]
  • Renal involvement with lymphoma occurs much more commonly with non-Hodgkin disease. The majority of patients have intermediate or high-grade lymphomas, most of them of B-cell origin.[casesjournal.biomedcentral.com]
Constitutional Symptom
  • Primary retroperitoneal lymphomas are a rare occurrence in clinical practice and their non-specific clinical presentation comprised primarily of constitutional symptoms.[symptoma.com]
Inguinal Hernia
  • The inguinal region demonstrated a complete indirect inguinal hernia with cystic consistency.[current-oncology.com]
Recurrent Epigastric Pain
  • Radionuclide emisssion tomography and ultrasonography were obtained on a patient with recurrent epigastric pain. In the pancreatic region an ultrasonic scan revealed a conglomerated mass.[ncbi.nlm.nih.gov]
Painful Ophthalmoplegia
  • Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic.[symptoma.com]
  • Abstract Numb chin syndrome (NCS) and painful ophthalmoplegia (PO) are neurological syndromes associated with the disturbance of certain cranial nerves and their downstream nerves.[jstage.jst.go.jp]
Numb Chin Syndrome
  • Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic.[symptoma.com]
  • Abstract Numb chin syndrome (NCS) and painful ophthalmoplegia (PO) are neurological syndromes associated with the disturbance of certain cranial nerves and their downstream nerves.[jstage.jst.go.jp]

Workup

Because the diagnosis is quite easily missed without a thorough workup, physicians must consider lymphomas in their differential diagnosis in the setting of unexplained constitutional symptoms. A properly conducted physical examination could reveal enlarged cervical lymph nodes during palpation of the neck and a mass during palpation of the abdomen. These findings require further workup through imaging studies. Plain radiography is not useful in the evaluation of soft tissues. For this reason, computed tomography (CT), often considered to be the diagnostic modality of choice, or magnetic resonance imaging (MRI) of the abdomen, thorax and pelvis are recommended [4] [5]. A well-defined homogenous mass in the midline is often the characteristic feature of a lymphoma on CT, and compression or even displacement of various anatomical structures, such as the aorta, vena cava or iliac vessels, may be noted [1] [4]. MRI, on the other hand, shows isointense (T1) or hyperintense (T2) images and a homogenous mass [1]. Imaging criteria are not sufficient to confirm lymphoma, especially when almost a quarter of all Hodgkin lymphomas present as a heterogeneous mass [1]. For this reason, a definite diagnosis is only made after performing a biopsy. Various approaches have been described in the literature, including CT-guided needle biopsy, nodal excision, and surgical resection [3] [6] [7] [8]. Through microscopic examinations, immunohistochemistry and molecular studies performed on the obtained sample, the exact subtype of lymphoma can be identified, which is essential for choosing optimal therapy [3].

Treatment

  • They’ll tell you how to cope during treatment, but life after cancer has it’s own battles.[pinterest.de]
  • It highlights our limited knowledge of the tumor biology and prominently demonstrates our limited available treatment options To cite this abstract: Pleat N, Andolina M, Panke T.[shmabstracts.com]
  • Secondary malignancies after treatment for indolent non-Hodgkin’s lymphoma: a 16-year follow-up study. Haematologica 2008;93:398–404.[current-oncology.com]
  • Treatment Treatment of retroperitoneal tumor treatment Department of Oncology: Department of Oncology Surgery Treatment: Surgical treatment Drug treatment Supportive therapy Radiation therapy Treatment cycle: 3-6 months Cure rate: 60% Commonly used drugs[healthfrom.com]
  • But treatment options for NHL are on the rise.[webmd.com]

Prognosis

  • Both infer a poor prognosis. Conclusions: This represents the first reported case of an EBV negative primary retroperitoneal extranodal NK/T‐cell lymphoma with bone marrow metastasis.[shmabstracts.com]
  • They are classified into 3 subgroups—germinal-centre B-cell-like, activated B-cell-like, and primary mediastinal dlbcl —according to the pattern of gene expression, with each having a different prognosis.[current-oncology.com]
  • References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis[amboss.com]
  • Complication Retroperitoneal tumor complications Complications of lymphoma The prognosis of malignant tumors is poor, the recurrence rate after resuscitation can be as high as 30% -50%, and the degree of malignancy increased with the recurrence of 5 years[healthfrom.com]
  • Prognosis Low-grade NHL types have a relatively good prognosis, with median survival as long as 10 years; however, they are usually not curable when advanced.[patient.info]

Etiology

  • The diagnosis is difficult to attain without an extensive clinical and radiological workup, whereas a biopsy is necessary to confirm hematologic malignancy and exclude other possible etiologies.[symptoma.com]
  • Etiology Secondary retroperitoneal fibrosis Malignancies and exposure to methysergide are the most common causes of secondary RPF ![amboss.com]
  • Causes (Etiology) of Retroperitoneal Fibrosis Unknown Etiology In 70% of patients with retroperitoneal fibrosis, the etiology of Ormond's disease is unclear.[urology-textbook.com]
  • Etiology Retroperitoneal tumor etiology Disease etiology: Etiology is unclear, retroperitoneal tumors are benign and malignant two major categories, about 60-80% of malignant tumors, common with liposarcoma, fibrosarcoma, fibrofibrous sarcoma and malignant[healthfrom.com]
  • Renal imaging plays a valuable role in determining the etiology of renal failure due to intrinsic or extrinsic obstruction of the urinary tract.[ukidney.com]

Epidemiology

  • Lymphomas constitute a heterogeneous group of diseases with epidemiologic associations.[current-oncology.com]
  • Epidemiology Epidemiological data refers to the US, unless otherwise specified. Etiology Secondary retroperitoneal fibrosis Malignancies and exposure to methysergide are the most common causes of secondary RPF ![amboss.com]
  • EPIDEMIOLOGY Retroperitoneal fibrosis is a rare disease.[uptodate.com]
  • Epidemiology of Retroperitoneal Fibrosis 40–60 years, male:female 2:1. Causes (Etiology) of Retroperitoneal Fibrosis Unknown Etiology In 70% of patients with retroperitoneal fibrosis, the etiology of Ormond's disease is unclear.[urology-textbook.com]
  • Furthermore, reduction of E2F1 expression led to decreased growth capacity in sBL cells in vitro. [20] Epidemiology According to the SEER (Surveillance Epidemiology and End Results) database, the yearly incidence of Burkitt lymphoma/leukemia in the United[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis[amboss.com]
  • One assumed mechanism of disease is an autoimmune process with periaortitis as the initial pathophysiological mechanism. Later, the inflammatory-fibrotic mass extends into the retroperitoneum and compresses the ureters.[urology-textbook.com]
  • […] and pathophysiologic mechanisms leading to the development of Burkitt lymphoma are not known.[emedicine.medscape.com]

Prevention

  • […] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer.[nccn.org]
  • Prevention Retroperitoneal tumor prevention Retroperitoneal tumor mainly from the retroperitoneal space, deep in the site, asymptomatic early, with the development of the tumor, before the waist pain or abdominal organs appear to oppress the symptoms[healthfrom.com]
  • The hiatus of the peritoneum for the ureters must be large enough to prevent iatrogenic ureteral compression. Omentum majus can be wrapped around the ureters for a safe separation to the retroperitoneal fibrosis.[urology-textbook.com]
  • Your surgeon will use a variety of intraoperative techniques to prevent lymphatic leak.[hopkinsmedicine.org]
  • Combination therapy must be avoided in young male patients to prevent the emergence of hepatosplenic lymphoma.[karger.com]

References

Article

  1. Rajiah P, Sinha R, Cuevas C, Dubinsky TJ, Bush WH Jr, Kolokythas O. Imaging of uncommon retroperitoneal masses. Radiographics. 2011;31(4):949-976.
  2. Fulignati C, Pantaleo P, Cipriani G, et al. An uncommon clinical presentation of retroperitoneal non-Hodgkin lymphoma successfully treated with chemotherapy: A case report. World J Gastroenterol. 2005;11(20):3151-3155.
  3. Chen L, Kuriakose P, Hawley RC, Janakiraman N, Maeda K. Hematologic malignancies with primary retroperitoneal presentation: clinicopathologic study of 32 cases. Arch Pathol Lab Med. 2005;129(5):655-660.
  4. Strauss DC, Hayes AJ, Thomas JM. Retroperitoneal tumours: review of management. Ann R Coll Surg Engl. 2011;93(4):275-280.
  5. CAI Y-L, XIONG X-Z, LU J, LIN Y-X, CHENG N-S. Non-Hodgkin’s lymphoma with uncommon clinical manifestations: A case report. Oncol Lett. 2015;10(3):1686-1688.
  6. Ichikawa S, Saito K, Suzuki T, Kimura J, Harigae H. Primary retroperitoneal diffuse large B-cell lymphoma presenting with numb chin syndrome and painful ophthalmoplegia. Intern Med. 2013;52(16):1813-1819.
  7. Tomozawa Y, Inaba Y, Yamaura H, et al. Clinical Value of CT-Guided Needle Biopsy for Retroperitoneal Lesions. Korean J Radiol. 2011;12(3):351-357.
  8. Chakrabarti I, Bhowmik S, Sinha MGM, Bera P. Ultrasound-guided aspiration cytology of retroperitoneal masses with histopathological corroboration: A study of 71 cases. J Cytol. 2014;31(1):15-19.

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Last updated: 2018-06-21 22:52