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Umbilical Endometriosis
UMBILICAL ENDOMETRIOSIS A Case Report MICHAEL F. BEIRNE, M.D., AND SAMUEL
w. BERKHEISER, M.D., SAYRE, PA.
(llrom the Department of Pathology at the G'Utlwie Clinic and Robert Packer Hospital)
NDOMETRIOSIS of the umbilicus is uncommon, and when discovered E usually provokes considerable discussion, particularly as to etiology. Survey of the literature reveals that since Villar first described this condition in 1886 about 108 cases have been reported. During the two menses preceding hospitalization, this 48-year-old white woman had experienced for the first time in her life some soreness of the umbilicus accompanied by a slight bloody discharge. This soreness and bleeding began and ended with the menstrual periods. Previous menses had always been normal and regular with no dysmenorrhea. There had been one normal pregnancy 12 years before. She had always been healthy and active and other past history was without significance. With the first episode the family physician cauterized the bleeding point of the umbilicus and on examination found a small pelvic mass. The second episode brought the patient to the hospital, at which time the positive physical fiudings were limited to the umbilicus and the pelvis. The umbilicus was slightly enlarged, definitely darker in color and of increased firm· ness. Pelvic examination revealed a tender mass 5 em. in diameter, in the right adnexa. 'l'he uterus was slightly irregular and was thought to contain fibroids. A bilateral salpingo-oophorectomy and hysterectomy were performed for fibromyomas of the uterus and endometriosis. There was extensive endometriosis involving the myome· trium, the tubes, and ovaries, with a chocolate cyst, 5 em. in diameter, of the right ovary. A small dermoid cyst, 1 em. in diameter, of the left ovary was also present. The umbilicus was removed intact and on section revealed several small cystic structures containing a dark, coffee-ground type of material. Microscopic examination of the tissues confirmed the diagnosis of pelvic endometriosis. Beneath the skin of the umbilicus there was a great deal of old hemorrhage, as well as numerous endometrium-like glands. These glands were surrounded by a well-defined dense cytogenic stroma characteristic of endometrial stroma; some of the glands contained old blood (Fig. 1).
Comment Endometriosis is defined by Novak1 as "the condition in which tissue resembling endometrium more or less perfectly is found aberrantly in various locations.'' An anatomical differentiation is made by some authors between internal endometriosis (adenomyosis) and external endometriosis. The former extends diffusely throughout the uterine wall, whereas the latter type is found on or near the peritoneal surface of the uterus, ovaries, pelvis, or elsewhere in the body. Isolated endometrial tissue, surrounded by endometrial stroma or smooth muscle, may be called an endometrioma. 895
896
.\rn . J. Obst. & Gynec. April, I
~mlometl'iosis occurs duriug· the sexually adiYe period of life, stat'ting afte1· pubet·ty and stopping- in most instances 111 1h e menopause.' .lVIost often it is found in the four·th and fifth decades. Ectopic endometrium does uot aLways participate. in menstruation; fre-
quently the intermenstrual eyclic changes Ht'e absent, the tissue being unable to respond to hormones! There are at least seven different theories concerning the pathogenesis of endometriosis; they are: (1) direct myometrial extension; (2) congenital theories; (3) serosal metaplasia; (4) implantation theory; ( 5) lymphatic metastasis; (6) hematogenous metastasis; (71 analogy to endometrial carcinoma.
Fig. I.-Ph otomicrograph r evealing the dark-staining, dense endometrium-like stroma su rround ing the glands, some of which contain old blood.
Since 1921, following Sampson 's 3 classic description of this condition, the etiology of endometriosis has been a subject of great dispute. Sampson advanced the theory that implantation of endom etrial particles in abnormnl areas was the r esult of retrograde menstruation. N ovak1 and others, on the other hand, have objected to Sampson's theory on the following grounds: (1) Retrograde menstruation, while it may occur, is a rare phenomenon, as contrasted with the great frequency of endometriosis. (2) It is difficult to believe that endometrium, which is dead when thrown off, eould enter the small uterine orifice of the tube, travel against the current, and still he capable of implanting itself and gTowing. (3) Experiments in which a
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UMBILICAL ENilOM:E'rRIOSIS
uteroabdominal fistula was created in monkeys, failed to show any development of endometrium in spite of the fact that menstrual blood was emptied freely into' the abdominal cavity. (4) Sampson's theory could not explain endometriosis in certain locations, such as that of the umbilicus. Most of the authors opposed to the implantation theory of Sampson favor the view that the aberrant endometrium has its origin from abnormal differentiation of the celomic epithelium from which all genital mucous membranes arise (the celomic or serosal metaplasia theory). This theory permits the development of endometrium wherever celomic epithelium or vestiges occur; these areas include the pelvic peritoneal surfaces, hernial sacs, and the umbilicus. Stimuli which have been thought to bring about such abnormal differentiation include inflammation and hormonal imbalance. 4 No single theory can adequately explain the pathogenesis of endometriosis, particularly of the cases reported occurring in the hand, thigh, axilla, lungs, and kidneys. Recently, however, Javert 6 advanced the composite theory which included concepts from the other theori<'s and may be summarized as follows: "(1) Endometrium is a benign tissue <:apable of extra-uterine spt·ead. It undergoes cyclical homeoplasia that may be a factor in the production of endometriosis. (2) Benign endometr-ial cells are capable of dissemination and metastasis along the same channels followed by endometrial adenocarcinoma. ( 3) The pattern of dissemination may be partial or complete in the following manner: direct extension into the myometrium, uterotubal junction and adjacent organs; exfoliation of viable (non-menstrual) endometrial cells into the vagina and through the fimbriated ends of the tubes; implantation of these cells on adjacent serosal surfaces; lymphatic metastasis to regional lymph nodes and adjacent organs; hematogenous metastasis to distant locations." Summary A case of umbilical endometriosis has been presented. The major theories explaining the pathogenesis of endometriosis have been enumerated and summarized.
References 1. Novak, R: Gynecological and Obstetrical Pathology, ed, ;;, Philadelphia, 19:i2, W. B. SaunderH Compan:v. 2. Ranney, B.: Internat. Abstr. Surg. 86: 313, 1948. 3. Sampson, J. A.: Arch. Burg. 3: 24;i, 1921. 4. Anderson, W. A.: Pathology, e
6. Javert, C. T.: Cancer 2: 39\J, 1949.
w. BERKHEISER, M.D., SAYRE, PA.
(llrom the Department of Pathology at the G'Utlwie Clinic and Robert Packer Hospital)
NDOMETRIOSIS of the umbilicus is uncommon, and when discovered E usually provokes considerable discussion, particularly as to etiology. Survey of the literature reveals that since Villar first described this condition in 1886 about 108 cases have been reported. During the two menses preceding hospitalization, this 48-year-old white woman had experienced for the first time in her life some soreness of the umbilicus accompanied by a slight bloody discharge. This soreness and bleeding began and ended with the menstrual periods. Previous menses had always been normal and regular with no dysmenorrhea. There had been one normal pregnancy 12 years before. She had always been healthy and active and other past history was without significance. With the first episode the family physician cauterized the bleeding point of the umbilicus and on examination found a small pelvic mass. The second episode brought the patient to the hospital, at which time the positive physical fiudings were limited to the umbilicus and the pelvis. The umbilicus was slightly enlarged, definitely darker in color and of increased firm· ness. Pelvic examination revealed a tender mass 5 em. in diameter, in the right adnexa. 'l'he uterus was slightly irregular and was thought to contain fibroids. A bilateral salpingo-oophorectomy and hysterectomy were performed for fibromyomas of the uterus and endometriosis. There was extensive endometriosis involving the myome· trium, the tubes, and ovaries, with a chocolate cyst, 5 em. in diameter, of the right ovary. A small dermoid cyst, 1 em. in diameter, of the left ovary was also present. The umbilicus was removed intact and on section revealed several small cystic structures containing a dark, coffee-ground type of material. Microscopic examination of the tissues confirmed the diagnosis of pelvic endometriosis. Beneath the skin of the umbilicus there was a great deal of old hemorrhage, as well as numerous endometrium-like glands. These glands were surrounded by a well-defined dense cytogenic stroma characteristic of endometrial stroma; some of the glands contained old blood (Fig. 1).
Comment Endometriosis is defined by Novak1 as "the condition in which tissue resembling endometrium more or less perfectly is found aberrantly in various locations.'' An anatomical differentiation is made by some authors between internal endometriosis (adenomyosis) and external endometriosis. The former extends diffusely throughout the uterine wall, whereas the latter type is found on or near the peritoneal surface of the uterus, ovaries, pelvis, or elsewhere in the body. Isolated endometrial tissue, surrounded by endometrial stroma or smooth muscle, may be called an endometrioma. 895
896
.\rn . J. Obst. & Gynec. April, I
~mlometl'iosis occurs duriug· the sexually adiYe period of life, stat'ting afte1· pubet·ty and stopping- in most instances 111 1h e menopause.' .lVIost often it is found in the four·th and fifth decades. Ectopic endometrium does uot aLways participate. in menstruation; fre-
quently the intermenstrual eyclic changes Ht'e absent, the tissue being unable to respond to hormones! There are at least seven different theories concerning the pathogenesis of endometriosis; they are: (1) direct myometrial extension; (2) congenital theories; (3) serosal metaplasia; (4) implantation theory; ( 5) lymphatic metastasis; (6) hematogenous metastasis; (71 analogy to endometrial carcinoma.
Fig. I.-Ph otomicrograph r evealing the dark-staining, dense endometrium-like stroma su rround ing the glands, some of which contain old blood.
Since 1921, following Sampson 's 3 classic description of this condition, the etiology of endometriosis has been a subject of great dispute. Sampson advanced the theory that implantation of endom etrial particles in abnormnl areas was the r esult of retrograde menstruation. N ovak1 and others, on the other hand, have objected to Sampson's theory on the following grounds: (1) Retrograde menstruation, while it may occur, is a rare phenomenon, as contrasted with the great frequency of endometriosis. (2) It is difficult to believe that endometrium, which is dead when thrown off, eould enter the small uterine orifice of the tube, travel against the current, and still he capable of implanting itself and gTowing. (3) Experiments in which a
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uteroabdominal fistula was created in monkeys, failed to show any development of endometrium in spite of the fact that menstrual blood was emptied freely into' the abdominal cavity. (4) Sampson's theory could not explain endometriosis in certain locations, such as that of the umbilicus. Most of the authors opposed to the implantation theory of Sampson favor the view that the aberrant endometrium has its origin from abnormal differentiation of the celomic epithelium from which all genital mucous membranes arise (the celomic or serosal metaplasia theory). This theory permits the development of endometrium wherever celomic epithelium or vestiges occur; these areas include the pelvic peritoneal surfaces, hernial sacs, and the umbilicus. Stimuli which have been thought to bring about such abnormal differentiation include inflammation and hormonal imbalance. 4 No single theory can adequately explain the pathogenesis of endometriosis, particularly of the cases reported occurring in the hand, thigh, axilla, lungs, and kidneys. Recently, however, Javert 6 advanced the composite theory which included concepts from the other theori<'s and may be summarized as follows: "(1) Endometrium is a benign tissue <:apable of extra-uterine spt·ead. It undergoes cyclical homeoplasia that may be a factor in the production of endometriosis. (2) Benign endometr-ial cells are capable of dissemination and metastasis along the same channels followed by endometrial adenocarcinoma. ( 3) The pattern of dissemination may be partial or complete in the following manner: direct extension into the myometrium, uterotubal junction and adjacent organs; exfoliation of viable (non-menstrual) endometrial cells into the vagina and through the fimbriated ends of the tubes; implantation of these cells on adjacent serosal surfaces; lymphatic metastasis to regional lymph nodes and adjacent organs; hematogenous metastasis to distant locations." Summary A case of umbilical endometriosis has been presented. The major theories explaining the pathogenesis of endometriosis have been enumerated and summarized.
References 1. Novak, R: Gynecological and Obstetrical Pathology, ed, ;;, Philadelphia, 19:i2, W. B. SaunderH Compan:v. 2. Ranney, B.: Internat. Abstr. Surg. 86: 313, 1948. 3. Sampson, J. A.: Arch. Burg. 3: 24;i, 1921. 4. Anderson, W. A.: Pathology, e
6. Javert, C. T.: Cancer 2: 39\J, 1949.