Collecting Duct Carcinoma of Kidney Differential Diagnosis of Neoplasms Involving the Renal Medulla

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Collecting Duct Carcinoma of KidneyDifferential
Diagnosis of Neoplasms Involving the Renal Medulla

• Merce Jorda, MD, PhD, and Murugesan Manoharan,
  MD
• (Pathology Case Reviews 200611 191196)

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Case Presentation

• A 61-year-old woman
• hematuria and abdominal discomfort.

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macroscopic examination

• Left kidney showed a central mass with necrosis
  measuring 12 cm in greatest dimension
• The tumor replaced the entire pyelocaliceal
  system, invaded the renal vein and the Gerota
  fascia, and metastasized to the adjacent adrenal
  gland

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Microscopic

• this neoplasm was characterized by a
  predominantly solid and canalicular pattern of
  growth with papillary areas
• Cell size ranged from small cuboidal to large,
  with eccentric nuclei, distinct nucleoli, and
  frequent mitoses.
• Hobnail cells were also identified

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Immunohistochemical stains

• high-molecular-weight keratin (HMWK) ()
• E-cadherin ()
• epithelial membrane antigen (EMA) ()
• Carcinoembryonic antigen (CEA) ()
• Ulex europaeus agglutinin-1 UEA-1 ()
• P63 / renal cell carcinoma antigen (RCC) / CD-10
  (-)

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DISCUSSION

• CDC, previously called Bellini duct carcinoma,
  was first described by Mancilla-Jimenez et al in
  1976.
• CDC is a rare renal neoplasm that originates from
  the distal segment of the collecting duct in the
  renal medulla pyramids known as the collecting
  ducts of Bellini

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DISCUSSION

• CDC is thought to arise from the ureteral bud or
  mesonephros, hence sharing the same origin as
  urothelial carcinomas.
• CDC accounts for 0.6 to 3 of all kidney
  carcinomas
• Of all renal epithelial neoplasms, CDC is the
  most aggressive, with early metastasis common at
  the time of clinical presentation

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Clinical Features

• 21 male to female ratio
• Mean age is 55 years,wide range (13 to 83)
• Association with chronic dialysis
• Abdominal pain, flank mass, and hematuria
• Imaging studies are usually suggestive of RPUC
  (pelvic/medullary location)

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Clinical Features

• Approximately 35 to 40 of patients have
  metastasis at presentation
• Common metastatic sites include regional lymph
  nodes, adrenal gland, bone, lung, and liver.
• More than 75 of patients die with disease within
  2 years of diagnosis

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Clinical Features

• Conventional treatments for renal carcinoma,
  including radiation, chemotherapy
• Immunotherapy are not effective

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Gross Pathology

• CDC is located in the central region of the
  kidney and often involves hilar structures.
• It ranges in size from 2.5 to 12 cm and is
  typically firm and gray-white, with infiltrating
  borders.
• It may present as multifocal, probably
  representing intrarenal metastasis.

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Microscopic Characteristics

• This neoplasm is characterized by tubular,
  tubulopapillary, or solid growth patterns with
  desmoplasia and inflammatory reaction.
• Small cyst formations may be present within
  folding of papillary growth.
• Sarcomatoid differentiation as a sign of
  dedifferentiation has also been described.

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Microscopic Characteristics

• Mucin production may be present
• Cytologically, CDC is composed of eosinophilic or
  basophilic high-nuclear grade cells with frequent
  hobnail pattern.
• (Papillary RCCs lack hobnail cells)

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Immunohistochemistry

• HMWK
• UEA-1
• E-Cad
• CK5/CK6/CK17 -
• CD10/15 -
• N-Cad -
• P63 -

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Cytogenetics

• Monosomy of chromosome 1 appears to be a constant
  finding in CDC.
• Other alterations described are monosomy of
  chromosomes 18 and 21, loss of the Y chromosome,
  and gains of chromosome 7, 12, 17, and 20.

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Molecular Alterations

• CDCs lose chromosomal arm 1q more frequently than
  other renal neoplasms, a feature shared with
  urothelial carcinomas.
• Loss of heterozygosity of chromosome 6p is
  observed in 50 of the CDCs.

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Molecular Alterations

• Loss of chromosome 3p, frequently seen in
  conventional RCCs, is a rare event in CDC. Only
  8 of CDC demonstrated alteration of the VHL gene
  (3p25-26).
• RCCs have not included CDC due to the rarity of
  this neoplasm

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Differential Diagnosis

• The principal differential diagnosis of CDC
  includes PC, RPUC, and metastatic carcinoma.
• Less frequently, other medullary renal neoplasms
  such as MC and TC may be part of the differential
  diagnosis

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Immunohistochemistry
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Papillary RCC

• a large neoplasm that may invade the renal
  medulla
• histologically characterized by the presence of
  fibrovascular cores with tumor cells arranged in
  papillary configuration.

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RPUC

• RPUC bears little morphologic resemblance to CDC
• occasionally extend to collecting ducts of the
  renal papillae
• immunohistochemical markers that may be useful

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Medullary carcinoma

• MC is a rare neoplasm first distinguished from
  CDC by Davis et al in 1995
• the strong association of this neoplasm with
  sickle cell trait is a helpful hint in the
  differential diagnosis.

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Tubulocystic carcinoma

• TC, also known as low-grade CDC
• Their immunohistochemical profile is similar to
  CDC
• Low nuclear grade and dilated tubules are key
  diagnostic features of TC

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Metastaric carcinoma

• Metastatic carcinomas, particularly those of
  gastrointestinal tract or lung origin, are part
  of the differential diagnosis.
• Immunohistochemistry for CDX-2 and TTF-1 may Be
  helpful
• Frequently multiple, well-circumscribed, and
  usually not associated with dysplastic changes of
  the collecting ducts.

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CONCLUSION

• CDC is a rare and aggressive renal neoplasm that
  shares biologic characteristics with carcinomas
  of urothelial origin.
• Because of origin in the renal medulla, the
  differential diagnosis is with other neoplasms
  that may involve the central area of the kidney.
• A correct diagnosis and distinction from other
  RCCs are imperative since prognosis and treatment
  modalities are different.