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 Lymphoma

  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. [symptoma.com]

  Retroperitoneal lymphe nodes: Mass around the aorta that biopsy proved to be a NHL (non Hodgkin's lymphoma) Foods That Fight Lymphoma Cancer Fighting Vegetables for Lymphoma [Article] Non-Hodgkin lymphoma: What you need to know. [pinterest.de]

  / [Radiologic diagnosis of non-Hodgkin's lymphoma in abdominal and retroperitoneal sites]. Portnoi, L M ; Golenkov, A K ; Shumskii, V I ; Roslov, A L ; Dibirov, M P ; Grishchenko, A B ; Fedosov, S N. [pesquisa.bvsalud.org]

  Further CT-guided biopsy was performed and non-Hodgkin lymphoma was diagnosed based on pathological results. RF is usually diagnosed through clinical presentation and imaging studies. [casereports.bmj.com]

  ICD-10-CM Diagnosis Code C85.9 Non-Hodgkin lymphoma, unspecified 2016 2017 2018 2019 Non-Billable/Non-Specific Code Applicable To Lymphoma NOS Malignant lymphoma NOS Non-Hodgkin lymphoma NOS Non-Hodgkin lymphoma, unspecified Approximate Synonyms Lymphoma [icd10data.com]

• Pain

  Abstract 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]

  Discussion Low back pain and radicular pain are frequent complaints, with the most common cause herniated intervertebral disc. [ard.bmj.com]

  Occasionally, patients also experience nonspecific symptoms including vague abdominal pain, weight loss, general malaise, and nausea. [cancertherapyadvisor.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]

• Soft Tissue Mass

  Large ill defined heterogeneous soft tissue mass is seen centered on the left posterior para renal space. [radiopaedia.org]

  At CT and MR imaging of angiomyolipomas, small tumors are homogeneous and larger tumors are heterogeneous soft-tissue masses that typically contain a large amount of macroscopic fat and hyperenhancing vascular soft tissue. [pubs.rsna.org]

  […] with thick septa (arrows) that show soft tissue attenuation. [de.slideshare.net]

  Typical imaging findings of renal lymphoma include multiple poorly enhancing masses, retroperitoneal tumors that directly invade the kidneys, a solitary mass, bilateral renal enlargement, and perirenal soft-tissue masses. [emedicine.medscape.com]

• Swelling

  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 [symptoma.com]

  The swelling of the nodes themselves is referred to as lymphadenopathy. Unlike other types of lymph node, retroperitoneal lymph nodes generally cannot be felt or seen when enlarged. [verywell.com]

  Features seen here include: - enlarged cervical nodes - hypoechoic nature of the nodes - prominent hilar vascularity Tuberculous lymphadenitis is a common finding and a very common cause of neck swellings in the pediatric age group in developing countries [ultrasound-images.com]

  CT of the abdomen Full history/Diagnosis is available below Diagnosis: Lymphoma with inferior vena cava obstruction Full history: 73 year old man presented with three month history of increasing swelling of the lower extremities and the scrotum. [gamma.wustl.edu]

  His chief complaint was a hard swelling in the lower abdomen for the last 3 months. The swelling was increasing at a rapid rate. He also complained of persistent lower abdominal pain for the last month. He had no bowel or urinary complaints. [jpma.org.pk]

• Weakness

  We present an unusual case in which a patient presenting with weakness and mild lower abdominal and pelvic pain was noted at sonography to have bilaterally enlarged ovaries with features similar to those of massive ovarian edema as described previously [ncbi.nlm.nih.gov]

  Temsirolimus is a possible treatment for relapsed or refractory mantle cell lymphoma but evidence is weak currently. [patient.info]

  Less commonly, non-Hodgkin's lymphoma may arise subdurally or within the spinal cord. 5 Spinal cord compression typically presents with back pain, leg numbness and tingling, radicular pain followed by extremity weakness, paresis and paralysis, but can [ard.bmj.com]

  Case Presentation A 54-year-old woman with a medical history of unprovoked lung and cerebral embolism presented with paresthesia and disabling weakness of the right hand that had persisted for 3 days. [link.springer.com]

  […] cell (Burkitt's) lymphoma include: A. endemic in central Africa B. strongly linked to HTLV-1 C. a low grade lymphoma D. most often a T cell lymphoma E. rarely has a leukemic phase You see a 12-year-old boy from the Shenandoah Valley because of vague weakness [med-ed.virginia.edu]

• Epigastric Pain

  Abstract 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]

• Recurrent Epigastric Pain

  Abstract 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]

• Hepatosplenomegaly

  Hepatosplenomegaly, adrenal gland enlargement and para aortic lymph nodes are other findings. 1 article features images from this case [radiopaedia.org]

  […] diagnosis of follicular lymphoma - histology enables an assessment of the tumour grade and the exclusion of transformation to a more aggressive histological subtype. [ 8 ] CT scan of the neck, chest, abdomen and pelvis: to detect enlarged lymph nodes, hepatosplenomegaly [patient.info]

  […] history-pneumoconioses-hilar & mediastinal nodes -manual labor-epitrochlear;axillary;inguinal c) exposure to animals (tularemia;catscratch) and drugs (Dilantin) physical examination with special attention to the location and extent of lymphadenopathy; potential hepatosplenomegaly [med-ed.virginia.edu]

  Hepatosplenomegaly with hepatic metastasis (white arrows). [21] Lymphoma is definitively diagnosed by a lymph node biopsy, meaning a partial or total excision of a lymph node examined under the microscope. [22] This examination reveals histopathological [en.wikipedia.org]

• Painful Ophthalmoplegia

  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]

  Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic. [symptoma.com]

• Suggestibility

  This might suggest a reasonable approach for young patients with large retroperitoneal tumors involving long segments of the ureter. Autotransplantation was performed only after a disease-free status was confirmed following chemotherapy. [ncbi.nlm.nih.gov]

  In our patient, radiologic features suggested a rather indolent variant. [current-oncology.com]

  Conclusions: Although the present series is small, our findings suggest that PRLBCL may represent a more heterogeneous group of tumors than previously thought, which merits further phenotypic and molecular studies to broaden the understanding of its histogenesis [moh-it.pure.elsevier.com]

  Most studies have suggested a 2 to 3:1 male-to-female predominance [ 5,8,11,12 ], although this is not reported in all studies [ 4 ]. [uptodate.com]

  Heterogeneous texture of the L3 vertebral body and pedicles as well as the anterior aspect of L4 suggestive of bone infiltration. Internal fixation of the lumbar spine at the levels of the involved lumbar vertebrae. [radiopaedia.org]

• Numb Chin Syndrome

  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]

  Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic. [symptoma.com]

• Pelvic Pain

  We present an unusual case in which a patient presenting with weakness and mild lower abdominal and pelvic pain was noted at sonography to have bilaterally enlarged ovaries with features similar to those of massive ovarian edema as described previously [ncbi.nlm.nih.gov]

• Asthenia

  Case Presentation The patient is a 54-year-old carpenter who had alcohol and tobacco habits presented with asthenia with low back pain in March 2018. [cureus.com]

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].

• Lymph Node Hyperplasia

  Retroperitoneal lymphadenopathy is typically seen in other nonneoplastic conditions such as mycobacterium avium-intracellulare infection (MAI) or Castleman’s disease (giant lymph node hyperplasia). [radiologykey.com]

They’ll tell you how to cope during treatment, but life after cancer has it’s own battles. [pinterest.de]

But treatment options for NHL are on the rise. [webmd.com]

Surgical Treatment of Retroperitoneal Fibrosis The surgical treatment (ureterolysis and intraperitoneal transfer of the ureter) is indicated for failure of medical treatment or if diagnosis by biopsy is unclear. [urology-textbook.com]

High-dose chemotherapy treatment with stem cell support. Radiotherapy. Temsirolimus is a possible treatment for relapsed or refractory mantle cell lymphoma but evidence is weak currently. [patient.info]

Surgical treatment (90%) followed by chemotherapy and radiotherapy are the methods of choice for treatment in most of the retroperitoneal tumours. Early diagnosis and multimodality treatment improves prognosis. [pjmhsonline.com]

References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis [amboss.com]

Burkitt lymphoma ) typically have a prognosis of weeks without treatment. [radiopaedia.org]

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]

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]

The classification of a lymphoma can affect treatment and prognosis. [en.wikipedia.org]

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]

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]

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]

The etiology, pathogenesis, clinical manifestations, and diagnostic evaluation of retroperitoneal fibrosis will be reviewed here. The treatment of retroperitoneal fibrosis is presented separately (see "Treatment of retroperitoneal fibrosis" ). [uptodate.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]

Lymphomas constitute a heterogeneous group of diseases with epidemiologic associations. [current-oncology.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]

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]

The pathophysiology of RPF is still poorly understood, but the autoimmune hypothesis has been validated by several studies. [cureus.com]

[…] 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]

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]

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.