Malakoplakia of the Colon Associated with a Villous Adenoma"

Report of a Case*

JEAN ROBERT, M.D., t R E A L LAGACE, M.D., + CLAUDE DELAGE, M.D.+

From the Department of Pathology, L'Hdtel-Dieu de Qudhec., lI, C6te dtz PalMs, Qudbec, Canada, GIN 2J6

MALAKOPLAKIA (from the Greek -- "soft plaque") is a granulomatous disease char- acterized by the presence of large histio- cytes, Hanseman 's cells, containing oval or spheric inclusions called "Michaelis-Gut- mann bodies." Since the first description of an example of the lesion found in the urin- ary bladder in 1902 by Michaelis and Gut- mann, TM other organs have been identified as the site of malakoplakia. Cases involving the testis, a, 7 the prostate, 3 the epididymis, b the kidney, n, 14 the retroperitoneum, is the skin,8, 12, is the stomach, 16 and the colon 1, 2,4, 15, 16 have been reported. Malako- plakia within the colon has been described either as an incidental finding in association with an adenocarcinoma or as a pr imary lesion.

This paper reports the association of malakoplakia with a villous adenoma. T o our knowledge, such an association has not yet been reported.

Report of a Case

A 75-year-old m a n sought medical a t tent ion in 1967 for weakness, weight loss, tenesmus and rectal incont inence. He had also blood and m u c u s in his stools. Rectoscopy showed a t u m o r located 5 cm from the anorectal junct ion. A biopsy was done and his topathologic examina t ion showed a ben ign villous adenoma . Because of old age and poor cardiac condit ion, the lesion was locally excised and cauterized. Two years later, in 1969, another local excision with cauterization was performed for recurrence of the villous adenoma; there were two

* Received for publication February 19, 1974. t Resident in Surgery. :~ Pathologist.

Dis. CoL & Rect. Sept.-Oct. 1974

more recurrences, in 1970 and 1971, each r equ i r i ng local excision. D u r i n g tha t per iod the pa t ien t h a d a myocardia l infarct ion. He was aga in seen at the end of 1971 because of ano the r recurrence of his rectal tumor . At tha t t ime, the histologic p ic ture was a little different. T h r e e m o n t h s later, the pa t ien t died of a second myocardia l infarct ion. Necropsy was not done.

Findings

Examination of the tissue specimen from the first excision showed a typical villous adenoma. The last specimen exanfined at the end of 1971 had also a characteristic villous pattern, but in addit ion showed an interesting finding: the connective tissue of the lamina propria was crowded with nu- merous closely packed macrophages or his- tiocytes (Fig. 1). These ceils were large, polygonal, and had a round nucleus and an eosinophilic, finely granular cytoplasm. PAS- and Von Kossa-positive round structures, sometimes surrounded by a light clear halo, were seen (Figs. 2 and 3). These ceils were identified as Hanseman's cells and the in- clusions as Michael i s -Gutmann bodies.

Serial sections from the four previous biopsies, 1969 through 1971, were reviewed, and morphologic features suggestive of malakoplakia were not found.

Comment

T h e presence of large histiocytes with Von Kossa, PAS- and Prussian blue-positive intracytoplasmic inclusions in the lamina propria of the rectal mucosa is virtually diagnostic of malakoplakia.

668 Volume 17 Number 5

Volume 17 M A L A K O P L A K I A O F T H E C O L O N 669 Number 5

FIG. 1. Part of an axis of the villous adenoma; the lamina propr ia is crowded by numerous histiocytes and a mixed cellular infiltration (hematoxylin and eosin; • 100).

Recent electron-microscopic studies have demonstrated that the foamy histiocytes con- sisted of macrophages,S, ~a and that Michael- is-Gutmann bodies in the cytoplasm of Hanseman's cells were limited by a single membrane, and were probably of lysosomal nature, i.e., cytosegresomes. Morphologic and histochemical studies have suggested that calcium and iron deposits and uniden- tiffed glycolipid substances were enclosed within lysosomes.~a

The histogenesis of Michaelis-Gutmann bodies remains obscure. They probably represent remnants of material which has been phagocytized by macrophages.

The etiology of malakoplakia is un- known. However, most of the cases in which malakoplakia is found in the genital and urinary tracts are associated with the pres- ence of Escherichia coli in the affected areas.

Very recently, intracellular bacteria were found in three cases of malakoplakia of the bladder. The correlative histolo~c and

ultrastructural studies showed a close rela- tionship between the bacteria, the mem- branous inclusions, and Michaelis-Gutmann bodies, It has been suggested that the histo- logic picture of malakoplakia might be initiated by the phagocytosis, the degrada- tion and host response to intracellular bac- teria, followed by calcification. Indeed, this observation reinforced the infectious etiol- ogy of the disease. In our case, it is also possible that bacteria, namely E. coli, could have caused the disease. The patient was submitted to four biopsies and electrocoagu- lative manipulations in the same area in the rectum. A carefnl search for typical histio- cytes with Michaelis-Gutmann bodies in the material of the first four excisions was nega- tive. Characteristic findings of malako- plakia were observed only in the last ex- cisional biopsy. From the above, it is tempting to imply that a local infection with E. coli could: have initiated the process of malakoplakia.

670 ROBERT, ET AL. Dis. Col. & Reef. Sept.-Oct. 1974

Flc. 2. Higher magnification of Figure 1, showing typical macrophages with inclusion bodies (hematoxylin and eosin; •

FiG. 3. Clusters of Hanseraan's cells containing Michaelis-Gutmann bodies, recognizable by the peripheral :dear .halo (hematoxylin and e0sin; • 450).

Volume 17 MALAKOPLAKIA OF THE COLON 671 Number 5

S u m m a r y

A case of m a l a k o p l a k i a associa ted w i t h a

v i l l ous a d e n o m a of the r e c t u m is desc r ibed .

T h e c y t o p l a s m of t he h i s t iocy tes of the

l a m i n a p r o p r i a was f i l led w i t h s t rongly PAS-

V o n Kossa, a n d P rus s i an b l u e pos i t ive ,

g ranu les , t he d i agnos t i c f ea tu re of ma la -

k o p l a k i a . F r o m recen t electron-microscopic f indings, i t is sugges ted t h a t m o r p h o l o g i c

f indings of m a l a k o p l a k i a are the r e su l t of

an i n f l a m m a t o r y process. T h e c a l c i u m de-

posi ts w h i c h are V o n Kossa-pos i t ive p rob-

ab ly cons t i t u t e the f o r m a l genesis of t he

d e g r a d a t i o n a n d hos t response to in t r a -

c e l l u l a r bac te r i a .

R e f e r e n c e s

I. Di Silvio TV, Bartlett EF: Malakoplakia of the colon. Arch Pathol 92: 167, 1971

2. Finlay-Jones LR, Blackwell JB, Papadimitriou JM: Malakoplakia of the colon. Am J Clin Pathol 50: 320, 1968

3. Goldman RL: A case of malacopIakia with in- volvement of the prostate gland. J Urol 93: 407, 1965

4. Gonzales-Angulo A, Corral E, Garcia.Torres R, et al: Malakoplakia of the colon. Gastro- enterology 48: 383, I965

5. Green WO Jr: Malacoplakia of the epididymis

(without testicular involvement): The first reported case. Arch Pathol 86: 438, 1968

6. Haukohl RA, Chinchinian H: Malakoplakia of the testicle: Report of a case. Am J Clin Pathol 29: 473, 1958

7. Lagac6 R: Malakoplakia du testicule. Laval MM 40: 276, 1969

8. Lederc J-L, Bernier L: Malacoplasie cutan6e. Union Med Can 101: 471, 1979

9. McClurg FV, D'Agostino AN, Martin JH, et al: Ultrastructural demonstration of intracellular bacteria in three cases of malakoplakia of the bladder. Am J Clin Pathol 60: 780, 1973

10. Michaelis L, Gutmann C: Uber Einschltisse in Blasentumoren. Z Klin Med 47: 208, 1902

11. Miller OS, Finck FM: Malacoplakia of the kidney: The great impersonator. J Urol 103: 712, 1970

12. Moore WM III, Stokes TL, Cabanas VY: Mal- akoplakia of the skin: Report of a case. Am J Clin Pathol 60: 218, 1973

18. Price HM, Hanrahan JB, Florida RG: Morpho- genesis oE calcium laden cytoplasmic bodies in malakoplakia of the skin: An electron microscopic study. Hum Pathol 4: 381, 1973

14. Smith BH: Malacoplakia of the urinary tract: A study of twenty-four cases. Am J Clin Pathol 43: 409, 1965

15. Terner JY, Lattes R: Malakoplakia of colon and retroperitoneum: Report of a case with a histochemical study of the Michaelis- Gutman inclusion bodies. Am J Clin Pathol 44: 20, 1965

16. Yunis EJ, Esterez JM, Pinzan GI, et al: Malako- plakia: Discussion of pathogenesis and report of three cases including one of fatal gastric and colonic involvement. Arch Pathol 83: 180, 1967