Adenomatoid tumor

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Adenomatoid tumor Microchapters

Overview

Pathophysiology

Causes

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Treatment

Differentiating Adenomatoid tumor from other diseases

Adenomatoid tumor
ICD-O: 9054/0
MeSH D018254

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.B.B.S.[2]

Synonyms and Keywords: Benign mesothelioma

Overview

Adenomatoid tumor is a rare benign mesothelial tumor affecting female and male genital tract (testicular adnexa). It generally presents in the genital tract, in regions such as the testis and epididymis.However, it also has been found in the pancreas.

Pathophysiology

  • Adenomatoid tumors are benign, solid extratesticular lesions that can originate from the epididymis, tunica vaginalis, or spermatic cord.[1][2][3]
  • They are the most common tumor of the epididymis.[4][5]
  • They occur more often in the lower pole than in the upper pole by a ratio of 4:1.[6][7][8]
  • Usually an incidental finding, adenomatoid tumors manifest as a painless scrotal mass, with the majority diagnosed in patients aged 20–50 years.[9][10][11]
  • They are typically unilateral and occur more frequently on the left side. When they grow noninvasively into the testicular parenchyma, they can simulate intratesticular disease.[12][13][14][15]

Gross pathology

  • 1 - 5 cm, well circumscribed solid tumor, adherent to testis / testicular adnexa
  • Cut surface may have small cystic spaces

Histopathology

  • No distinct growth pattern
  • Unencapsulated, cuboidal to flat cells forming cords that are either epithelial-like or form channels with dilated lumina simulating vessels
  • Cells have acidophilic cytoplasm with cytoplasmic vacuoles
  • Nuclei lack nucleoli
  • Mitoses and necrosis are usually absent
  • Intervening stroma may have smooth muscle and elastic fibers, desmoplastic quality and inflammatory cells
  • Rarely, tumor may extend into testicular parenchyma or even rarer, be totally inside the testis[5][13]
  • Patterns have been described but most tumors show a mixture of:[16][17][18][19][20]
    • Adenomatoid (tubular): glandular pattern with cytoplasmic vacuoles giving a signet ring appearance
    • Angiomatoid (canalicular): cells have a more endothelial appearance
    • Solid (plexiform): cells have a more epithelial appearance
    • Cystic (mixed): rare; tumor is in form of cystic spaces lined by cuboidal to flattened lining epithelium

Cytology

  • Smears are moderately cellular with sheets of monotonous round to oval cells showing indistinct cell borders and moderate to abundant pale cytoplasm with vacuolations [8][21]
  • Nuclei are eccentric in location but regular with inconspicuous nucleoli[22][3][15]

Immunostaining

  • Adenomatoid tumor stains positive for:[10]
    • Calretinin
    • Pan cytokeratins (EMA, AE1 / AE3, Cam5.2)
    • CK5 / 6
    • CK7 (variable, focal to diffuse)
  • Adenomatoid tumor stains negative for:
    • Vascular / endothelial markers (CD31, CD34)
    • CEA
    • HBME1
    • Factor VIII Ag
    • MOC31 / BerEP4
    • B72.3
    • CD15
    • Ulex europeus agglutinin I
    • Germ cell markers including:
      • OCT3 / 4
      • SOX2
      • AFP
      • PLAP
      • CD117
      • CD30
      • NANOG

Electron microscopy

  • Prominent microvilli
  • Desmosomes
  • Tonofilaments associated with dilated intercellular spaces

Causes

  • Mesothelial origin based on immunohistochemical features and ultrastructural studies [23]
  • Similar tumor in spermatic cord, ejaculatory duct, fallopian tube, uterus

Epidemiology and Demographics

  • 30% of testicular adnexal tumors, 73% of benign tumors of testicular adnexa in study of 314 tumors[24]
  • Most commonly affects individuals in third to fifth decade of life with mean age of invlovement being 36 years
  • Rarely can affect the two extremes of age:
    • A case report of adenomatoid tumor in a 5 year old boy[25]
    • A case report of adenomatoid tumor in a 70 year old man[26]
  • White race is more prone to develop adenomatoid tumor
  • Males are more likely to develop adenomatoid tumor comparative to females

Risk Factors

  • Schimmelpenning syndrome is associated with the presence of multiple adenomatoid odontogenic tumors[27]
  • Wilms tumors 1 WT1 gene has been implicated in the formation of the adenomatoid tumors[28]
  • Chronic myeloid leukemia (CML) patients on imatinib therapy may present with adenomatoid tumor as an incidental finding

Natural History, Complications and Prognosis

  • Uniformly benign behavior, no reports of malignant transformation

Diagnosis

History and Symptoms

Common sites of involvement

  • Sites involved by adenomatoid tumor are mentioned in the table below:
Sites involved by Adenomatoid tumor
Site Organs involved
Male genital tract[29][30][31][32][33][34][35][36][37][22][6][38][11][13][39][40] Common
Rare
Female genital tract[41][42][43][44][45][44][46][47][48][49][50][51][52]
Extragenital areas [53][54][55][56][57][58][27][59][60][61][62][63][64][65][66][67][68][69][70]

Symptoms

Presents as:

  • An incidental finding (asymptomatic for several years)
  • A solid, well circumscribed, slowly growing scrotal mass
  • Painless enlargement (normal scrotal skin and surrounding adenexa)
  • Pain (often)
  • Small in size usually ranging from 0.5–5.0 cm (rarely larger exceeding 2 cmm3)[71]
  • Benign (even if it extends into testis)

Laboratory findings

  • No specific laboratory finding; however, negative markers for germ cell tumor are helpful in excluding a germ cell malignancy

Ultrasound findings

  • On an ultrasound, they appear as a solid, homogeneous, extratesticular mass with variable echogenicity (hyperechoic or hypoechoic).[72]
  • USG reveals a relatively hypo, iso to hyperechoic mass at lower pole of epididymis, not distorting the testis

MRI

  • Commonly, MRI demonstrates low signal intensity relative to the testicular parenchyma on T2-weighted images.
  • MRI can aid in determining the paratesticular origin of the lesion.
  • Adenomatoid tumors enhance after administration of gadolinium contrast material.


T2
T2
STIR
T1 pre contrast
T1 post GAD


Pathological and radiological comparison of adenomatoid tumor of testis at other anatomic sites
Organ Gross appearance Histopathology Immunohistochemical staining Radiological features
Liver (cystic type) Cystic spaces having following characteristics:
  • Various sizes
  • Contain RBCs and colloid like matrix
  • Lined with cuboidal to low columnar and flattened epithelioid cells
  • Surrounded by collagenous stroma
  • Epithelioid cells having small to large vacuoles, eosinophilic cytoplasm and round to oval nuclei
  • Fine collagenous bands constituting the tumor border
  • No invasion into the liver parenchyma
Stains positive for: MRI characteristics of adenomatoid tumor of liver are as follows:
  • Hypointense on T1 weighted images
  • Hyperintense on T2 weighted images
  • Hyperintense on Gd enhanced images
Mesocolon and omentum
  • Tubules and anastomosing channels
  • Lined by cuboidal or flattened cells with eosinophilia
  • Uniform oval to round nuclei having vesicles
Stains positive for: CT characteristics of adenomatoid tumor of mesocolon and omentum are as follows:
  • Hypervascular tumor between uterus and rectum
  • Multiple small nodules in pelvic cavity
  • Well-circumscribed hypodense mass within the pancreas
Adrenal gland (tubules and glands) Appears as either:
  • Interconnecting tubules and glands\
  • Lined with plump epithelioid cells having plentiful eosinophilic cytoplasm to flat mesothelial like cell
  • Contains adipose tissue, lymphoid aggregated and mucin-producing areas
Stains positive for:

Stains negative for:

CT shows:
  • A small mass in the adrenal gland

MRI shows:

  • A small mass with no surrounding invasion
Pancreas (solid variant, usually cystic type)
  • Mixed spindle cells and tubules
  • Lined with attenuated cuboidal cells
Stains positive for:

Stains negative for:

CT of adenomatoid tumor of pancreas shows:
  • A well-circumscribed hypodense mass within the pancreas
Uterus
  • Gland like spaces
  • Lined by a single layer of cuboidal cells with prominent nuclei
  • May displace fibromuscular stroma or
  • Infiltrate diffusely with scattered clusters cells
Stains positive for: MRI characteristics of adenomatoid tumor of uterus are as follows:
  • Resembles leiomyoma without any degeneration
  • Low signal intensity with clear margins on T2 weighted and Gd- DTPA enhanced T1 weighted images
  • Isointensity on T1 weighted images
  • Patterns similar to normal myometrium in dynamic MRI
Ovary
  • Cuboidal to flat cells forming tubules and solid cords
  • Surrounded by patchy fibroid or myxoid stroma containing aggregates of inflammatory cells and smooth muscle fibers
  • Tubules with small cystic dilations
Stains positive for:

Stains negative for:

CT shows:
  • An ovarian lesion having septa and small solid central portion

Treatment

  • Complete excision
    • Radical orchidectomy
    • Laparoscopic excision of uterine adenomatoid tumor [51][50]
  • Frozen section examination may prevent unnecessary radical orchiectomy[73][74]
  • Paratesticular tumors are rare tumors that are difficult to diagnose preoperatively and therefore, many patients are subjected to inguinal orchiectomy. However, radical orchiectomy can be avoided as the diagnosis of paratesticular tumor can be made on the basis of clinical suspicion, findings of tumor markers and radiological tests.

Differentiating Adenomatoid tumor from other diseases

  • Adenomatoid tumor must be differentiated from:
    • Malignant mesothelioma (HBME1+, mitoses and necrosis)
    • Metastatic adenocarcinoma (positive for one or more of CEA, PSA, MOC31 / BerEP4 and CD15 are useful)
    • Papillary cystadenoma of epididymis

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