Case Report 807

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Radiological Category
Principal Modality (1) Principal Modality (2)
Gastrointestinal
Ultrasound
Computer Tomography
Case Report 807
Submitted by
Modupe Adeyefa, M.D.
Faculty reviewer
Varaha Tammisetti, M.D., The University of Texas
Medical School at Houston
Date accepted
11 April, 2011
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Case History

• 20 year old male with a three month history of
  back pain relieved by Aleve but the pain has
  progressively worsened. He went to his primary
  care physician where a right lower quadrant mass
  was palpated. An abdominal ultrasound was
  performed which showed two large retroperitoneal
  masses. He presented about a week later to
  Memorial Herman Hospital because of worsening
  abdominal pain and syncope.

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Radiological Presentations

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Radiological Presentations

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Radiological Presentations

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Radiological Presentations

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Radiological Presentations

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Radiological Presentations

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Radiological Presentations

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Ultrasound Two large heterogeneous echogenic
retroperitoneal masses.CT Large
intraperitoneal hemoperitoneum and large
sentinel clot adjacent to the necrotic,
hypervascular nodal masses in lower abdomen.
There are 3 large, centrally necrotic,
peripherally enhancing masses in the
retroperitoneum, the largest in the pelvis and
measures 9.58x8.8 cm. Smaller similar masses are
noted further superiorly .
Findings and Differentials
Findings
Differentials

• Lymphoma
• Retroperitoneal Metastases with tumor associated
  hemorrhage/ hemoperitoneum
• Sarcoma
• Retroperitoneal Hemangiopericytoma

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Discussion

• Lymphoma
• Accounts for the 10-15 of all childhood cancer
  which arises from constituent cells of the immune
  system or from precursors. Nodal and splenic
  involvement are more common in Hodgkin disease
  whereas extranodal involvement is more common in
  Non Hodgkin lymphoma. Both can involve the
  retroperitoneum. It appears as a mantle of soft
  tissue adenopathy surrounding the aorta, inferior
  vena cava, involving the para-aortic, aortocaval
  and retrocaval nodal groups. CT findings are
  enlarged nodes greater that 1.5 cm in the short
  axis involving bilateral retroperitoneal nodal
  chains. Encasement of the root of the mesentery
  and the superior mesenteric artery by a
  lymphomatous mass originating from multiple
  enlarged and confluent lymph nodes may produce
  the so called sandwich sign. They may displace
  aorta from the spine. The attenuation of nodes on
  CT is similar to muscle.
• Hemoperitoneum from pathologic splenic rupture is
  rarely associated with lymphoma in which case the
  sentinel clot would be seen in the perisplenic
  region. In this patient the sentinel clot is seen
  surrounding the lymph nodes and the lymph nodes
  are necrotic with hypervascularity, both of which
  are not common features associated with lymphoma.

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Discussion

• Retroperitoneal Metastasis with tumor associated
  hemorrhage
• Spontaneous hemoperitoneum rarely occurs in the
  absence of trauma, a surgical or interventional
  procedure, or anticoagulation therapy. In such
  cases, the possibility of the rupture of an
  un-identified neoplasm must be excluded. Although
  the occurrence is uncommon, any primary or
  metastatic tumor can rupture and bleed into the
  peritoneal cavity. The most common causes in this
  setting would be ruptured hepatocellular
  carcinoma or hepatocellular adenoma. However, no
  hepatic lesions are identified and sentinel clot
  is seen away from the liver. Pathologic splenic
  rupture may occur as a complication of a viral
  infection, including infection by
  cytomegalovirus, malaria, or Epstein-Barr virus
  a congenital disease a metabolic abnormality
  such as Gaucher disease or amyloidosis and,
  rarely, a neoplastic process such as
  hemangiomatosis, angiosarcoma, leukemia, or
  lymphoma. However, the sentinel clot is not in
  the perisplenic region and there is no
  splenomegaly.
• The spontaneous rupture of a metastatic lesion in
  a solid organ or lymph node is rare but usually
  results in massive hemoperitoneum. Lung
  carcinoma, renal cell carcinoma, and melanoma are
  the metastatic lesions that can cause
  hemoperitoneum.

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Discussion

• Sarcoma Malignant primary retroperitoneal tumor
  arising from various elements of primitive
  mesenchyme, urogenital ridge or embryonic
  remnants. The best diagnostic clue on imaging is
  a large heterogenous mass of fat and soft tissue
  attenuation displacing the retroperitoneal
  structures or viscera. The location is usually in
  the peri/paranephric region. They are classified
  into four types, liposarcoma, leiomyosarcoma,
  fibrosarcoma or rhabdomyosarcoma.
• Liposarcoma is composed of adipose tissue with
  attenuation values greater than 20 hounsfied
  unit. They are poorly marginated encapsulated
  masses with/without calcification. On CT they
  have hetero/homogenous enhancement with a lack of
  prominent vessels.
• Leiomyosarcoma is the second most common and are
  composed mostly of smooth muscle. They are
  hypervascular and contain feeding vessels. They
  may appear as large solid masses with hypoechoic
  cystic and necrotic remnants.
• Fibrosarcoma and malignant histiocytoma are the
  most common soft tissue sarcomas in adults.
• Rhabdomysarcoma are striated muscle tumors . They
  are isodence to muscle with ill defined margins
  on non enhanced CT and are heterogenously
  enhancing on contrast enhanced CT.

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Discussion

• Retroperitoneal Hemangiopericytoma are
  hypervascular neoplasm arising form pericytes. In
  a study by Goldman et al, these tumors were
  typically large and less frequently in pelvic
  retroperitoneal space than in abdominal
  retroperitoneal. The most distinctive radiologic
  finding is hypervascularity, Other findings
  includes well defined margins and necrosis with
  nondistinctive amorphous calcifications.

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Additional Images

Multiple pulmonary nodules with halo of ground
glass haziness halo sign
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Discussion

• The halo sign, refers to a zone of ground glass
  attenuation surrounding a pulmonary nodule or
  mass on CT images and they are associated with
  hemorrhagic nodules, examples include
• Angio-invasive aspergillosis is a form of tissue
  invasion either angioinvasive or airway invasive
  typically in patients with neutropenia or
  impaired neutrophil function. Imaging findings
  include single or multiple nodules, a central
  hypodensity due to infarction also known as
  hypodense sign. The halo sign is also present
  which shows a large bulls eye surrounded by
  smaller rim ground glass opacification. An air
  cresent sign is a late sign and follows recovery
  of neutrophils.
• Hemorrhagic/ hypervascular metastases such as
  from choriocarcinoma, renal cell carcinoma,
  angiosarcoma or melanoma. The nodules may be
  sharply defined. These findings mirrors chest
  x-ray findings with metastases predominate in the
  outer 1/3 of the mid and lower lung zones.
  Hematogenous nodules may have visible feeding
  artery or the halo sign.

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Discussion

• Wegener granulomatosis A form of vasculitis
  that affects the lungs, kidneys and other organs.
  The best diagnostic clue are multiple cavitary
  lung nodules and large airwary narrowing. CT
  findings are similar to metastases as the nodules
  can have feeding vessels. Peripheral wedge shaped
  consolidation from infarct may be present. Some
  nodules have the CT halo sign from surrounding
  hemorrhage. The trachea and bronchi are
  concentrically thickened, either focal or long
  segments.
• Post transplant lymphoproliferative disorder
  (PTLD) it is a relatively uncommon complication
  of both solid organs and allogeneic bone marrow
  transplantation. In most cases, PTLD is
  associated with Epstein Barr virus infection of B
  cells, either as a consequence of a reactivation
  of the virus posttransplantation or from primary
  EBV infection acquired from the donor. CT
  findings include airspace consolidation and
  nodular opacities with hazy margins.
• Pulmonary Kaposi sarcoma Acquired immune
  deficiency syndrome related multicentric
  neoplasm with propensity to involve skin, lymph
  nodes, GI tract and lungs. Thoracic
  manifestations include bronchovascular bundle
  thickening progressing to coalescent, flame
  shaped perihilar consolidation, poorly defined
  nodules, reticular and nodular opacities with
  basilar predominance. There is also marked
  enhancement following intravenous contrast.

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Additional Images

Heterogeneous right testicular mass with cystic
focus, few tiny calcifications, vascularity and
irregular margins.
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Based on the findings from the retroperitoneal
masses, positive halo sign on CT and a testicular
mass, the diagnosis is more likely an hemorrhagic
or hypervascular metastases from a
non-seminomatous germ cell tumor such as from
choriocarcinoma or mixed germ cell
tumor.
Differential
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METASTATIC MIXED OR NONSEMINOMATOUS GERM CELL
TUMOR (NSGCT) SUCH AS CHORIOCARCINOMA

Diagnosis
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Pathology report from biopsied retroperitoneal
masses. - Immunohistochemical stains for
Pancytokeratin, Vimentin, Beta-HCG, Alpha
Feto-Protein, CD30, Placental Alkaline
Phosphatase, and Desmin were applied to the cell
block. The positive stains are Beta HCG with the
rest being negative. This supports the diagnosis
of a choriocarcinoma. - Morphologically and
immunohistochemically, the tumor was a
choriocarcinoma. However the retroperitoneal
mass is large, therefore a mixed germ cell tumor
cannot be excluded.
Discussion
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Testicular Cancer is relatively uncommon in the
United States with approximately 5500 cases per
year. These tumors arises in males of nearly any
age and may be of germ cell or non germ cell
origin. There are three main types, germ cell
tumors, non germ cell tumor and extragonadal
tumors.
Discussion
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Choriocarinoma is a rare germ cell tumor and in
its pure form , it is seen in less than 1 of
patients, but it occurs in mixed cell tumors in
8 of cases. It typically affects younger men and
unlike other cancers, choriocarcinoma
metastasizes hematogenously, with the testicular
primary tumor often small or even burnt out. It
metastasizes early via hematogenous routes to the
lung, liver, and brain, among others. This tumor
represents the most common malignancy in men
between the ages of 15-35 years of age. The tumor
responds poorly to radiation and chemotherapy and
carries high mortality rate. Patients with mixed
germ cell tumors with choriocarinoma fair better
than those with pure choriocarcinoma tumors, but
a high human chorionic gonadotropin (gt50,000
IU/L) portends a poor prognosis with at 5 year
survical rate of 48. Surgery is usually limited
to radical orchiectomy.
Diagnosis
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After four rounds of chemotherapy, the patient
had a right radical orchiectomy which showed a
mixed germ cell tumor consisting of mature
teratoma (approximately 95) and seminoma (less
than 5). No residual choriocarcinoma was seen
which indicates excellent response to therapy.

Discussion
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Goldman SM, Davidson AJ, Neal j. Retroperioteneal
and pelvic hemangiopericytomas clinical,
radiologic and pathologic correlation.
Radiology. July 1988, 168 13-17Meghan Lubner,
MD, Christine Menias, MD, Creed Rucker, MD,
Sanjeev Bhalla, MD, Christine M. Peterson, MD,
Lisa Wang, MD and Brett Gratz, MD. Blood in the
Belly CT findings of Hemoperitoneum.
Radiographics. January 2007. Volume 27,
109-125Paolo Toma, MD, Claudio Granata, MD,
Andrea Rossi, MD, Alberto Garaventa, MD.
Multimodality Imaging of Hodgkin Disease and
Non-Hodgkin Lymphoma in Children. Radiographics.
September-October 2007. Volume 27(5)
1335-1347Woodward et al. From the Archives of
the AFIP, Tumors and Tumorlike Lesions of the
Testis Radiologic and Pathologic Correlation.
January 2002. Radiographics, Vol 22,
189-216.Statdx.comwww.emedicine.com

References