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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].

Soft Tissue Mass
  • Large ill defined heterogeneous soft tissue mass is seen centered on the left posterior para renal space.[radiopaedia.org]
  • CT CT abdomen with contrast Large pre-vertebral homogenous retroperitoneal soft tissue mass lesion measuring 7.5 x 12.7 x 11 cm.[radiopaedia.org]
Hodgkin's Disease
  • Epidemiology NHL is more than five times as common as Hodgkin's disease.[patient.info]
Camping
  • Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, et al. Primary Sjögren syndrome in Spain, clinic and immunologic expression in 1010 patients. Medicine. 2008; 87 :210–9. . [ PubMed ] [ Google Scholar ] 3.[ncbi.nlm.nih.gov]
Falling
  • Thus intermediate lymphomas fall between the low and high grade lesions in length of survival.[med-ed.virginia.edu]
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]
Colic
  • Rare signs include a painful ophthalmoplegia, numb chin syndrome (both signs of a systemic malignancy) and renal colic.[symptoma.com]
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
  • 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]
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]
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]
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]

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]
  • The NCCN Templates are not exhaustive and do not represent the full spectrum of care or treatment options described in the NCCN Guidelines or the NCCN Compendium or include all appropriate approaches or combinations of drugs or biologics for the treatment[nccn.org]
  • References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis[amboss.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]
  • References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis[amboss.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]
  • Lymphoma: Clinical - Non-Hodgkin Here we will discuss the clinical presentation,staging, treatment and prognosis different for non-Hodgkin lymphomas.[med-ed.virginia.edu]

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]
  • 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]
  • Lymphoma: Non-Hodgkin -Clinical-Etiology The etiologic causes of non-Hodgkin lymphoma are usually not specifically identified. Viruses, chronic antigenic stimulation, and immunosuppression have been implicated in several types of lymphoma.[med-ed.virginia.edu]

Epidemiology

  • 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 NHL is more than five times as common as Hodgkin's disease.[patient.info]
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]

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]
  • Your surgeon will use a variety of intraoperative techniques to prevent lymphatic leak.[hopkinsmedicine.org]
  • First, the T cell immune surveillance system is suppressed or destroyed either by drugs (given to prevent rejection of the transplant) or by a virus (ie. HIV). Second, the B cell population may be antigenically stimulated to proliferate ( ie.[med-ed.virginia.edu]

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