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Granular cell myoblastoma of multicentric origin
GRANULAR CELL MYOBLASTOMA OF MULTICENTRIC ORIGIN* WILLIAM H . GUTSTEIN, M.D. Brooklyn, New York N 1949 Murphy, Dockerty and Broders in a comprehensive review of the literature, employing specified criteria, reported a total of 219 accepted cases of myoblastoma and added ten cases of their own . Since then several cases have been reported, bringing the total to nearly NO."'" In 1934 Klemperer reported six cases ." Of these, Case 2 was one in which two tumors were concurrently present . one in the skin of the left lateral region of the abdomen and the second in the skin of the suprapubic region . The intervening period has seen no additional reports of multiple benign myoblastomas . It is believed . that the present case is the second such case to be reported . Moreover, the contralateral locations involved emphasize the multicentric origins of the neoplasms .
I
CASE REPORT
On August 8, 1950, a thirty-eight year old Negro woman was admitted to Sydenham Hospital with a history of noticing a "tumor" in the region of the lower abdomen . For approximately a year previously the patient had noticed a lengthening of her menstrual periods from a duration of four to eight days . Past history disclosed that the patient had had a "tumor" removed from the left axilla in 1942 . The nature of this lesion could not be ascertained . Physical examination of the abdomen revealed a large, smooth, firm tumor rising out of the pelvis reaching to the umbilicus . On pelvic examination a preoperative diagnosis of multiple myomas of the uterus was made . On examination of the skin and subcutaneous tissues three small, firm, movable tumors were found under the skin . The first of these was located in the left anterior axillary wall and was 3 to 4 cm . in diameter . The overlying skin was reddened . The second was approximately of the same size and situated along the right
anterior axillary line at the level of the fifth intercostal space encroaching on the outer border of the breast . A third enlargement was reported as consisting of a small firm tumor, palpable in the right axilla, but was thought to be a lymph node . Laboratory data were as follows : hemoglobin 12 .5 gm. per too cc . ; red blood cells 4,100,000 per cu. mm . ; white blood cells it,8oo per cu . mm. Differential smear : polymorphonuclear leukocytes 58 per cent, transitional cells 12 per cent, lymphocytes 27 per cent, eosinophils 3 per cent . On urinalysis examination a trace of albumin was found . At laparotomy it was noticed that the pelvis was filled with fibrous adhesions, and the right ovary was cystic and enlarged . Subserous and intramural myomas of the uterus were present . Hysterectomy and right oophorectomy were performed . Following this the subcutaneous tumors in the left anterior axillary wall and right chest wall were removed . Both of these were thought to be fibromas at operation . The postoperative course was uneventful and the patient left the hospital eight days following operation . It was not possible to obtain a follow-up study on this patient . The pathologic description is confined to the subcutaneous nodules that were removed . Grossly the specimen removed from the right chest consisted of two portions of white "fibrous tissue," both irregular in shape . The larger portion measured 3 cm . in the direction of its longest diameter and the smaller, approximately 1 .5 cm . It was remarked that this tissue had the gross appearance of a fibroma . The tumor removed from the left anterior axillary wall was a somewhat oval-shaped mass measuring 4 by 3 .5 by 2 cm . One surface was covered by skin containing a sparse distribution of hair . The mass was of firm consistency, and its cut surface was white and presented a whorl-like pattern . A thin capsule was described
* From the Department of Pathology, Sydenham Ilospital, New York, N . Y.
bo 0
American Journal of Surgery
Gutstein- -Granular Cell Myoblastoma
701
Fts, T . Section from right anterior chest wall . Tuner (T) seen below encroaching upon normal breast tissue (13) . I lematoxylin and eosin, X 90 . Fn ; . 2 . High magnification of tumor in right chest wall . Hematoxylin and eosin, X 360 . Fie .3 . Section from left anterior axillary wall . Overlying epithelium slightly atrophic . Tumor in lower central portion of photograph extending into corium from below . The cyst-like spaces arc artifactual . I-lematox,vlin and eosin, X 90 . Fic . 4 . liigh magnification of Figure 3 . Note granularity of cytoplasm and similarity to Figure 2 .
investing the periphery . It was remarked that this tumor also had the gross appearance of a fibroma . Microscopically the sections of the specimen removed from the right chest wall revealed the presence of a tumor mass encroaching upon essentially normal breast tissue . (Fig . 1 .) No invasive properties were demonstrated and the entire mass was composed of cells proliferating in a somewhat orderly fashion . For the most part the tumor was composed of compact bundles of cells which were polyhedral or oval in shape, with deep-staining round or oval nuclei . The cytoplasm stained intensely eosinophilic and appeared granular. (Fig . 2,) The cell bundles were frequently syncytial in structure and separated from each other by fine strands of collagen in which fibroblasts were occasionally seen . Fat stains were negative. With van Gieson's stain the cell bundles stained orange-yellow
A4ay, 1953
whereas the intercellular connective tissue strands were deep crimson . The sections of the specimen removed from the axillary wall of the contralateral side exhibited a neoplastic proliferation of cells pushing into but not invading the subcutaneous tissue from below . (Fig . 3 .) The overlying epithelium disclosed a very slight degree of atrophy but otherwise was unremarkable . High magnification examination of the cells revealed an appearance altogether similar to that of the tumor in the right chest wall . (Fig . 4 .) In both specimens the diagnosis was compatible with that of the uniform type of granular cell "myoblastoma ." COMMENTS
The histologic classification of these tumors has undergone metamorphosis since Abrikossoff's original description of four groups' to the classification into two histologic groups as
702
Gutstein-Granular Cell Myoblastoma
noted in the article by Murphy, Dockerty and Broders .1 In accordance with the latter the present case is compatible with the uniform type as distinguished from the pleomorphic type . The localization of the tumor is diverse and apparently may be encountered in almost any part of the body . It has appeared in as widely scattered areas as the bronchus and urinary bladder .',' The degree of malignancy has been estimated to be in the neighborhood of I i per cent.10 Such tumors are considered to be rhabdomyosarcomas and may undergo metastasis . Histologically these are usually of the pleomorphic type . Shortly after the tumor was given notice by Abrikossoff, the view was expressed that myoblastomas were tumors which were basically rhabdomyomas in an early stage of developments Since then there has been much speculation about the nature of this tumor, whether it is myomatous in origin or not .' Recent histochcmical analysis indicates that these tumors may be fibroblastic in origin rather than myoblastic .4 Opinion is still somewhat divided as to whether myoblastoma is a true neoplasm,' or whether it represents a degenerative change . The present case of multiple myoblastomas may fit equally well into either of these two views . The unusual feature of the case presented in this article is the multicentric origin of the tumor . The report by Klemperer together with the present one reveals that myoblastoma has in common with other tumors the feature of multiplicity . The eighteen-year lapse in time together with the frequency of two cases in 300, or approximately 0 .7 per cent, shows however that such an occurrence is rare . The two tumors, the one removed from the right anterior chest wall and the other from the left anterior axillary fold, while contralaterally disposed are alike in their histology . The possibility of monocentric origin followed by contralateral metastasis of a malignant neoplasm is unlikely on the grounds of the benign cytologic appearance of both tumors, although at least one observer has reported metastasis from a lesion of this type ." A reasonable assumption is that each tumor arose independently from the subcutaneous tissues of its
respective side . The patient was not aware of a time relationship in the appearance of the two masses and a chronologic sequence for the appearance of one of the tumors before the other could not be established . However, it would seem that such a sequence is more probable than the simultaneous origin of both neoplasms . It is moreover noteworthy that a third tumor was removed from the left axilla eight years before the present admission . Information as to the nature of this lesion was not obtainable, but speculation based on the present findings suggests that this too may have been a myoblastoma . The small firm tumor described in the right axilla was not removed at operation, but was thought to be a lymph node . SUMMARY The second case of granular cell myoblastoma of multicentric origin is reported . The time lapse between reports is eighteen years . REFERENCES I . KERN, A . B ., KAUFMAN, J . J., COMBES, F . C . Granular cell myoblastoma ; report of a case simulating granuloma inguinale . Arch . Dermot . er Syph ., 62 : 109, 1950. 2 . MURPHY, G . W., DOCKERTY, M . B . and BRODERS, A . C . Myoblastoma . Am. J. Path ., 25 : 1157, U9493 . ABRIKOSSOFF, A . Ueber Myome, ausgehend von der quergestreiften Willkurlichen Muskulature . Vircbows Arch . J. path. Anat ., 260 : 215, 1926 . 4 . PEARSE, A . G. E . The histogenesis of granular-cell myoblastoma . J. Path . o' Bacl ., 62 : 351, 1950 . 5 . SADLER, W . P . and DocKERTY, M . B . Malignant myoblastoma vulvae . Am . J. Ohst . c Gynec., 61 : 1047, 1951 . 6 . KRAUS, A . R., MELNICK, P . J . and WEINNBERG, J . M . Myoblastoma of the bronchus . J . Thoracic Surg ., 17 : 382, 1948. 7 . RAvicn, A ., STOUT, A . P . and RAvICH, R . A . Malignant granular cell myoblastoma involving the urinary bladder . Ann . Sure ., 121 : 361, 1945 8 . SCHIRMER, R. Ober cin Myoblastenmyom zusammen mit Cancroid der Z unge . Beitr . z . path . Anat. u .z . allg . Path ., 89 : 613, 1932 . 9. OGDEN, W . W . and HERTZOG, A . J . Granular cell myoblastomas, report of three cases, New Orleans D4. " S. J ., 103 : 378, 1951 To . ORR, T . G . J . Myoblastoina, case report . Ann . Surg., 122 : 122, 1945 II . KHANOLKAR, V. R . Granular cell inyoblastoma . Am . J . Path,, 23 : 721, 1947 12 . KLEMPERER, P. Myoblastoma of the striated muscle . Am . J. Cancer, 20 : 324, 1934 . American Journal of Surgery
I
CASE REPORT
On August 8, 1950, a thirty-eight year old Negro woman was admitted to Sydenham Hospital with a history of noticing a "tumor" in the region of the lower abdomen . For approximately a year previously the patient had noticed a lengthening of her menstrual periods from a duration of four to eight days . Past history disclosed that the patient had had a "tumor" removed from the left axilla in 1942 . The nature of this lesion could not be ascertained . Physical examination of the abdomen revealed a large, smooth, firm tumor rising out of the pelvis reaching to the umbilicus . On pelvic examination a preoperative diagnosis of multiple myomas of the uterus was made . On examination of the skin and subcutaneous tissues three small, firm, movable tumors were found under the skin . The first of these was located in the left anterior axillary wall and was 3 to 4 cm . in diameter . The overlying skin was reddened . The second was approximately of the same size and situated along the right
anterior axillary line at the level of the fifth intercostal space encroaching on the outer border of the breast . A third enlargement was reported as consisting of a small firm tumor, palpable in the right axilla, but was thought to be a lymph node . Laboratory data were as follows : hemoglobin 12 .5 gm. per too cc . ; red blood cells 4,100,000 per cu. mm . ; white blood cells it,8oo per cu . mm. Differential smear : polymorphonuclear leukocytes 58 per cent, transitional cells 12 per cent, lymphocytes 27 per cent, eosinophils 3 per cent . On urinalysis examination a trace of albumin was found . At laparotomy it was noticed that the pelvis was filled with fibrous adhesions, and the right ovary was cystic and enlarged . Subserous and intramural myomas of the uterus were present . Hysterectomy and right oophorectomy were performed . Following this the subcutaneous tumors in the left anterior axillary wall and right chest wall were removed . Both of these were thought to be fibromas at operation . The postoperative course was uneventful and the patient left the hospital eight days following operation . It was not possible to obtain a follow-up study on this patient . The pathologic description is confined to the subcutaneous nodules that were removed . Grossly the specimen removed from the right chest consisted of two portions of white "fibrous tissue," both irregular in shape . The larger portion measured 3 cm . in the direction of its longest diameter and the smaller, approximately 1 .5 cm . It was remarked that this tissue had the gross appearance of a fibroma . The tumor removed from the left anterior axillary wall was a somewhat oval-shaped mass measuring 4 by 3 .5 by 2 cm . One surface was covered by skin containing a sparse distribution of hair . The mass was of firm consistency, and its cut surface was white and presented a whorl-like pattern . A thin capsule was described
* From the Department of Pathology, Sydenham Ilospital, New York, N . Y.
bo 0
American Journal of Surgery
Gutstein- -Granular Cell Myoblastoma
701
Fts, T . Section from right anterior chest wall . Tuner (T) seen below encroaching upon normal breast tissue (13) . I lematoxylin and eosin, X 90 . Fn ; . 2 . High magnification of tumor in right chest wall . Hematoxylin and eosin, X 360 . Fie .3 . Section from left anterior axillary wall . Overlying epithelium slightly atrophic . Tumor in lower central portion of photograph extending into corium from below . The cyst-like spaces arc artifactual . I-lematox,vlin and eosin, X 90 . Fic . 4 . liigh magnification of Figure 3 . Note granularity of cytoplasm and similarity to Figure 2 .
investing the periphery . It was remarked that this tumor also had the gross appearance of a fibroma . Microscopically the sections of the specimen removed from the right chest wall revealed the presence of a tumor mass encroaching upon essentially normal breast tissue . (Fig . 1 .) No invasive properties were demonstrated and the entire mass was composed of cells proliferating in a somewhat orderly fashion . For the most part the tumor was composed of compact bundles of cells which were polyhedral or oval in shape, with deep-staining round or oval nuclei . The cytoplasm stained intensely eosinophilic and appeared granular. (Fig . 2,) The cell bundles were frequently syncytial in structure and separated from each other by fine strands of collagen in which fibroblasts were occasionally seen . Fat stains were negative. With van Gieson's stain the cell bundles stained orange-yellow
A4ay, 1953
whereas the intercellular connective tissue strands were deep crimson . The sections of the specimen removed from the axillary wall of the contralateral side exhibited a neoplastic proliferation of cells pushing into but not invading the subcutaneous tissue from below . (Fig . 3 .) The overlying epithelium disclosed a very slight degree of atrophy but otherwise was unremarkable . High magnification examination of the cells revealed an appearance altogether similar to that of the tumor in the right chest wall . (Fig . 4 .) In both specimens the diagnosis was compatible with that of the uniform type of granular cell "myoblastoma ." COMMENTS
The histologic classification of these tumors has undergone metamorphosis since Abrikossoff's original description of four groups' to the classification into two histologic groups as
702
Gutstein-Granular Cell Myoblastoma
noted in the article by Murphy, Dockerty and Broders .1 In accordance with the latter the present case is compatible with the uniform type as distinguished from the pleomorphic type . The localization of the tumor is diverse and apparently may be encountered in almost any part of the body . It has appeared in as widely scattered areas as the bronchus and urinary bladder .',' The degree of malignancy has been estimated to be in the neighborhood of I i per cent.10 Such tumors are considered to be rhabdomyosarcomas and may undergo metastasis . Histologically these are usually of the pleomorphic type . Shortly after the tumor was given notice by Abrikossoff, the view was expressed that myoblastomas were tumors which were basically rhabdomyomas in an early stage of developments Since then there has been much speculation about the nature of this tumor, whether it is myomatous in origin or not .' Recent histochcmical analysis indicates that these tumors may be fibroblastic in origin rather than myoblastic .4 Opinion is still somewhat divided as to whether myoblastoma is a true neoplasm,' or whether it represents a degenerative change . The present case of multiple myoblastomas may fit equally well into either of these two views . The unusual feature of the case presented in this article is the multicentric origin of the tumor . The report by Klemperer together with the present one reveals that myoblastoma has in common with other tumors the feature of multiplicity . The eighteen-year lapse in time together with the frequency of two cases in 300, or approximately 0 .7 per cent, shows however that such an occurrence is rare . The two tumors, the one removed from the right anterior chest wall and the other from the left anterior axillary fold, while contralaterally disposed are alike in their histology . The possibility of monocentric origin followed by contralateral metastasis of a malignant neoplasm is unlikely on the grounds of the benign cytologic appearance of both tumors, although at least one observer has reported metastasis from a lesion of this type ." A reasonable assumption is that each tumor arose independently from the subcutaneous tissues of its
respective side . The patient was not aware of a time relationship in the appearance of the two masses and a chronologic sequence for the appearance of one of the tumors before the other could not be established . However, it would seem that such a sequence is more probable than the simultaneous origin of both neoplasms . It is moreover noteworthy that a third tumor was removed from the left axilla eight years before the present admission . Information as to the nature of this lesion was not obtainable, but speculation based on the present findings suggests that this too may have been a myoblastoma . The small firm tumor described in the right axilla was not removed at operation, but was thought to be a lymph node . SUMMARY The second case of granular cell myoblastoma of multicentric origin is reported . The time lapse between reports is eighteen years . REFERENCES I . KERN, A . B ., KAUFMAN, J . J., COMBES, F . C . Granular cell myoblastoma ; report of a case simulating granuloma inguinale . Arch . Dermot . er Syph ., 62 : 109, 1950. 2 . MURPHY, G . W., DOCKERTY, M . B . and BRODERS, A . C . Myoblastoma . Am. J. Path ., 25 : 1157, U9493 . ABRIKOSSOFF, A . Ueber Myome, ausgehend von der quergestreiften Willkurlichen Muskulature . Vircbows Arch . J. path. Anat ., 260 : 215, 1926 . 4 . PEARSE, A . G. E . The histogenesis of granular-cell myoblastoma . J. Path . o' Bacl ., 62 : 351, 1950 . 5 . SADLER, W . P . and DocKERTY, M . B . Malignant myoblastoma vulvae . Am . J. Ohst . c Gynec., 61 : 1047, 1951 . 6 . KRAUS, A . R., MELNICK, P . J . and WEINNBERG, J . M . Myoblastoma of the bronchus . J . Thoracic Surg ., 17 : 382, 1948. 7 . RAvicn, A ., STOUT, A . P . and RAvICH, R . A . Malignant granular cell myoblastoma involving the urinary bladder . Ann . Sure ., 121 : 361, 1945 8 . SCHIRMER, R. Ober cin Myoblastenmyom zusammen mit Cancroid der Z unge . Beitr . z . path . Anat. u .z . allg . Path ., 89 : 613, 1932 . 9. OGDEN, W . W . and HERTZOG, A . J . Granular cell myoblastomas, report of three cases, New Orleans D4. " S. J ., 103 : 378, 1951 To . ORR, T . G . J . Myoblastoina, case report . Ann . Surg., 122 : 122, 1945 II . KHANOLKAR, V. R . Granular cell inyoblastoma . Am . J . Path,, 23 : 721, 1947 12 . KLEMPERER, P. Myoblastoma of the striated muscle . Am . J. Cancer, 20 : 324, 1934 . American Journal of Surgery