No.1252
Esophagus, stomach and duodenumof a monkey

LSI Medience Corporation

Case History
Specimens: Esophagus, stomach and duodenum
Animal:Cynomolgus monkey, male, 6-year-oldHistory:

The animal was bred normally without any treatment. This
animal showed wasting, diarrhea and vomiting (food residue),though no decrease in food consumption. Then the animal waseuthanized due to deteriorating general condition.

Gross :
findings

No gross abnormalities were observed in digestive tract.
In other organs, small thymus and gelatinous change in femoral
bone marrow were observed.

Body Weight

The animal showed chronic wasting from 4 years
and 2 months of age (weighed 3.9 kg) until 6 yearsof age (2.4 kg). Food consumption did not decreaseduring housing. Diarrhea and vomiting (foodresidue) were also observed a month before
necropsy.

4.5
4
3.5
Body weight(kg)

3
2.5
24

4.5

5Age(years old)

5.5

6

Smooth muscle cells, Vein, Esophagus

Pigment granules are remarkably observed in the smooth muscle cells of the vein in the adventitia of theesophagus.
Pigment granules were also observed in the veins of the stomach and duodenum.

Smooth muscle cells, Artery, Esophagus

Pigment granules are observed in the smooth muscle cells of the artery in the adventitia of the esophagus.
Pigment granules were also observed in the artery of the stomach.

Smooth muscle cells, Muscularis propria, Esophagus

Pigment granules are observed in the smooth muscle cells of the muscularis propria in the esophagus. They
are not observed in the striated muscles of the esophagus.
Pigment granules were also observed in the muscularis propria of the stomach and duodenum.

Smooth muscle cells, Muscularis mucosa, Stomach

Pigment granules are observed in the smooth muscle cells of the muscularis mucosa in the stomach (arrows).
Pigment granules were also observed in the muscularis mucosa of the stomach and duodenum.

Pigment granules, Vein

Various-sized brown or basophilic pigment granules are distributed diffusely within the cytoplasm (arrows)
or observed around the nuclei (arrow heads) of the smooth muscle cells.

SchmorlPAS

Ziehl Neelsen

Dark green

Positive

Dark red

Berlin Blue

Kossa

Eosin onlyNegative

Negative

Yellowish brown

Staining Result of Pigment Granules
Lipofuscin

Pigment granules of the caseHE

Yellowish brown

Brown or Basophilic

Schmorl

Dark green

Dark green

PAS

Positive

Positive

Ziehl Neelsen(Dark) Red

Dark red

Berlin Blue

Negative

Negative

Kossa

Negative

Negative*Eosin only

Yellowish brown

Yellowish brown*Eosin only staining was conducted because the pigment was suspected to be modified by basophilic substance.

Although the pigment granules were basophilic in HE stain, they were identified
to be lipofuscin by special stains and eosin only stain.

Histopathological Diagnosis
in the Submitted Specimens
Lipofuscin Deposition in Smooth Muscle Cells
of Esophagus, Stomach and Duodenum,
Predominantly in Veins
食道・胃・十二指腸の平滑筋細胞へのリポフスチン沈着，静脈を主体とする

Pigment Distribution
Vein

ArteryMuscularis propria

Muscularis mucosa

Esophagus

+++

++

++

+

Stomach+++/-++Duodenum+/-

-

++

-

Jejunum+++

-

Ileum

++

-++

-

Cecum+---Colon+/--+-Rectum++/-++-Urinary bladder--++Kidney++++Lung++-Pancreas+++++Thymus+++-Epididymis++-

Mesenteric Lymph node+++++

Mandibular Lymph node++-Systemically, pigment granules deposited in the smoothmuscle cells of the organs listed in the table; however, there
was no tendency of deposition in specific organ systems.
The pigment granules were observed remarkably in bloodvessels, predominantly in veins. In neurons, myocardiocytes
and hepatocytes known as common sites of lipofuscin
deposition, no pigment granules were observed.

Diagnosis
Smooth Muscular Lipofuscinosis,Predominantly in Veins
平滑筋のリポフスチノーシス,静脈を主体とする

Intestinal Lipofuscinosis (humans)• In humans, intestinal lipofuscinosis is known as a very rare
condition characterized by lipofuscin deposition,
predominantly in smooth muscle cells of the digestive tract.
• Gross discoloration of the intestinal wall is observed. It iscaused by deposition of lipofuscin on the muscularis propria
and muscularis mucosa of any section of the digestive tract.
• Deposition in vascular smooth muscle cells is reported in a few
patients, but deposition is mainly in the digestive tract unlike
the present case.
• Intestinal lipofuscinosis is associated with various
malabsorption syndromes that cause vitamin E deficiency.
Pathogenesis is unknown.
Bialas M et al. 2013. Pol J Pathol 63(3): 228-231.

Stamp GWH, Evans DJ. 1987. J Clin Pathol 40: 798-802.

Case -Summary• A 6-year-old male cynomolgus monkey showed chronic wasting without any decrease in foodconsumption.
• No gross abnormalities were observed in necropsy expect for changes secondary to wasting.
• Microscopic examination revealed pigment granule deposition in systemic smooth muscle cells.
• Pigment granules stained brown or basophilic with HE stain. Special stains and eosin only stain
identified the pigment granules as lipofuscin.
• Unlike intestinal lipofuscinosis in humans, the pigment granules distributed systemically in bloodvessels and in the digestive tract, predominantly in veins.
• To our knowledge, this is the first report on smooth muscular lipofuscinosis predominantly observedin the veins of primates.

Pigment Granules•

Most pigment granules stained brown or basophilic, but rarely, eosinophilicgranules were observed in the smooth muscle cells in HE stain.

•The eosinophilic granules did not indicate the same staining characteristics
as brown or basophilic granules by special stains.
Mainly observed

Eosinophilic

Brown

BasophilicPigment granules (Eosinophilic), Muscularis propria, Duodenum

Rarely, eosinophilic granules are observed (arrows) in the smooth muscle cells of the duodenum in HE stain.
Few eosinophilic granules were also observed in the veins and muscularis propria of the esophagus andstomach.

Pigment granules

Ultrastructurally, the pigment granules are consistent with rounded or irregularly shaped residual bodies. Various amounts ofribosomes are observed in the background.
Three types of the cluster of residual bodies are shown in the next slide.

・Mostly disrupted organelles ormyelinoid membranes.
・Dense-granular materials with orwithout membrane.

・Irregularly-shaped residual bodies
sometimes including remnants oforganelles or myelinoid membranes.

・Uniformly round residual bodies.

・Few ribosomes.

・Mild to moderate amount ofribosomes.

・Large amount of ribosomes.

These might suggest different stages ofmaturation of the lipofuscin observedin this study.
It is suggested that brown to basophilic
granulesinalight
microscopereflect
・Mostly disrupted organelles or・Irregularly-shaped residual bodies
・Uniformly round residual bodies.
sometimes
including remnants ofmyelinoid membranes.
theamountofribosomes.
organelles or myelinoid membranes.
・Dense-granular materials with orwithout membrane.

・Few ribosomes

・Mild to moderate amount ofribosomes.

・Large amount of ribosomes.