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]

  Non-Hodgkin's Lymphoma is a group of blood cancers that affect the lymphatic system. The many types of lymphoma can be divided into two categories: Hodgkin’s lymphoma and Non-Hodgkin’s lymphoma (NHL). [pinterest.de]

  Abstract We describe a case of non-Hodgkin's lymphoma arising in the peripancreatic retroperitoneal lymph node and extending into the pancreatic parenchyma. [ncbi.nlm.nih.gov]

  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]

  Computer tomographic evaluation of digestive tract non-Hodgkin lymphomas. J Gastrointestin Liver Dis 2007;16:315–19. 11. Ekstrom–Smedby K. Epidemiology and etiology of non-Hodgkin lymphoma—a review. Acta Oncol 2006;45:258–71. 12. [current-oncology.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]

• 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]

• 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]

• Xerostomia

  A non-smoking 76-year-old female patient was diagnosed with SS in 2003 based on the American-European Consensus Group Criteria for Sjögren’s syndrome ( 3 ) (xerostomia, xerophthalmia, a positive Schirmer test ( 3 (N 4000–12,900) (neutrophils 2560/mm 3 [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]

• Lacrimation

  To the Editor, Sjögren’s syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands, leading to a progressive destruction of these glands, and is frequently accompanied by systemic symptoms [ncbi.nlm.nih.gov]

  Conclusions: Medial canthoplasty combined with CDCR is an effective surgical method for treatment of patients with medial telecanthal deformity and lacrimal drainage system obstruction. [ingentaconnect.com]

• 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]

• 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]

• Hyperactivity

  NHL, several considerations have to be taken into account in our case: first, the presence of various autoantibodies, such as the RF and anti-SSA/SSB antibodies, as well as hypergammaglobulinemia and positive circulating cryoglobulins, reflects B-cell hyperactivity [ncbi.nlm.nih.gov]

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].

• Normocytic Anemia

  A CBC, BMP, PTT, and PT/INR revealed only a normocytic anemia (Hb 11.7, MCV 87). An Abdominal/Pelvis CT scan discovered a 9x8x11cm mass in the left retroperitoneum (Figure 1 ). [shmabstracts.com]

• Lymphocytic Infiltrate

  The evolution from benign lymphocytic infiltration characteristic of SS to malignant NHL is probably a multistep process, the underlying molecular events of which are still unknown ( 2, 5, 6 ). [ncbi.nlm.nih.gov]

  Right lower corner shows the neoplastic lymphocytes infiltrating between smooth muscle cells. Occasional inflammatory cells are seen. Hematoxylin and eosin stain, 400 magnification. (B) CD20 staining of cells, consistent with B-cell origin (400 ). [current-oncology.com]

  ・左鎖骨上腫大リンパ節 Maligant lymphoma 免疫染色によりDiffuse large B cell lymphoma ・皮疹および胃の発赤隆起病変からはatypical lymphocytic infiltrationのみ。 ・臨床病気IVE ・Hyper-CVAD（エンドキサン、アドリアマイシン、オンコビン、デカドロン）にて化学療法導入。 その後、R-CODOX-M/R-IVACを1コース施行。 [trc-rad.jp]

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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.
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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.