Granular Cell Myoblastoma of the Cystic Duct A Case Associated with Hydrops of the Gallbladder
George J. Reul, Jr, MD,’ Houston, Texas Pedro A. Rubio, MD, Houston, Texas Norman L. Berkman, MD, Houston, Texas
Granular cell myoblastoma is a benign, well circumscribed nodular lesion that has been found most frequently on the skin and subcutaneous tissue, the oral cavity, and the tongue. Recently, LiVolsi et al [I] have also described its location in the breast, female reproductive tract, pituitary gland, gastrointestinal tract, bronchi, and, only rarely, the biliary system. This report is the first to describe true hydrops of the gallbladder caused by a granular cell myoblastoma obstructing the cystic duct. Case Report The patient, a thirty-nine year old black woman, was admitted to the Methodist Hospital to undergo elective cholecystectomy after nonvisualization of the gallbladder. She had had typical symptoms of gallbladder disease, including pain in the right upper abdominal quadrant and epigastrium with radiation to the back and infrascapular area, for approximately six years. The pain was unrelieved by antacids, frequently caused her to awaken at night, and was becoming increasingly severe. She denied having had jaundice. Physical examination demonstrated severe dextroscoliosis of the thorax but was otherwise noncontributory. Results of laboratory studies including liver function tests were likewise within normal limits. From the Cora and Webb Mading Department of Surgery. Baylor College of Medicine and the Methodist Hospital, Houston, Texas. * Present address and address for reprint requests: Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
Volume 129, May 1975
Elective cholecystectomy was performed on April 5, 1973. The gallbladder was tense and distended but without palpable stones; however, a nodule was palpable in the cystic duct approximately 1 cm from its entrance into the common duct. The common duct and pancreas appeared normal. Routine cholecystectomy was carried out. White mucoid material was present in the distended gallbladder distal to a small nodule that appeared to arise in the valves of Hiester and that totally obstructed the cystic duct. From frozen section study of the nodule, a diagnosis of granular cell myoblastoma of the cystic duct was made. On further pathologic examination, the gallbladder measured 8 by 4 by 3 cm. The serosa was intact, and the wall of the gallbladder measured 3 mm in thickness. A thick layer of dense mucous material covered the gallbladder mucosa. In the terminal portion of the cystic duct, the wall appeared grossly thickened up to 5 mm in diameter. The thickening was eccentric. The mass involved an 8 mm portion of the terminal cystic duct. Microscopic examination demonstrated a granular cell tumor that involved the submucosa and muscularis of the cystic duct. The distal margin of the cystic duct was uninvolved. Microscopic section showed almost complete obliteration of the cystic duct. (Figures 1 and 2.1
Comment Granular cell myoblastoma is usually a benign tumor although occasional cases of malignancy have been reported [2-41. LiVolsi et al [I] have stated
that
some
malignant
variants
of granular
583
Reul, Rubio, and Berkman
Figure 1. Granular ceil tumor (lower half of photomicrograph) bulges into the lumen, causing marked stenosis of the cystic duct (hematoxyiin and eosin stain; original magnification X 45).
Figure 2. Tumor ceils in detail show characteristic granuiar cytoplasm and uniform, dark, ovokt nuclei (hematoxyiin and eosin stain; original magnification X 675).
cell tumors may actually represent multifocal lesions since granular cell tumors are frequently multicentric [4]. There is much controversy concerning the precursor cell of the tumor. Murray [5] has proposed that the granular cell tumor arises from a striated muscle cell, from which the term, “myoblastoma,” was derived [5]. However, others have suggested a neural origin since these cells resemble Schwann cells on electron microscopy [4-61. Granular cell myoblastoma of the biliary tract has been reported infrequently, there being only eleven cases in the literature [I]. All cases have occurred in females. Six lesions were found in the cystic duct, four in the common duct, and one in the common hepatic duct. Most patients had a preoperative diagnosis of cholelithiasis and nine of the eleven patients underwent surgery with no mortality or recurrence. In the remaining two patients the diagnosis was made at autopsy. Obstructive jaundice was found in two patients. Severe hepatic failure with hepatic coma resulted in one patient with obstruction of the common bile duct in whom the lesion was found at autopsy.
Summary
584
The first known case of true hydrops of the gallbladder that resulted from granular cell myoblastoma is reported. Granular cell tumors are benign multicentric tumors that should be treated by total excision so that free margins are assured. These tumors are rarely found in the biliary tract; however, they may cause complications of biliary tract obstruction.
References 1. LiVolsi VA, Perzin KH, Badder EM, Price JB, Porter M: Granular cell tumors of the bitiiry tract. Arch Pathol 95: 13, 1973. 2. Crawford ES, DeBakey ME: Granular cell myoblastoma: two unusual cases. Cancer 6: 786, 1953. 3. Ross RC, Miller TC, Foote FW: Malignant granular cell myoblastoma. Cancer5 112, 1952. 4. Svejda J. Horn V: A disseminated granular cell pseudotumor: so-called metastasizinggranularcell myoblastoma. J Pathol76: 343, 1958. 5. Murray MR: Cultural characteristics of three granular cell myoblastomas. Cancer 4: 857, 195 1. 6. Pearse AGE: The histogenesis of granular cell myoblastoma (7 granular cell perineural fibroblastoma). J Patio/ 62: 351, 1950.
The American Journal
of Suqery
George J. Reul, Jr, MD,’ Houston, Texas Pedro A. Rubio, MD, Houston, Texas Norman L. Berkman, MD, Houston, Texas
Granular cell myoblastoma is a benign, well circumscribed nodular lesion that has been found most frequently on the skin and subcutaneous tissue, the oral cavity, and the tongue. Recently, LiVolsi et al [I] have also described its location in the breast, female reproductive tract, pituitary gland, gastrointestinal tract, bronchi, and, only rarely, the biliary system. This report is the first to describe true hydrops of the gallbladder caused by a granular cell myoblastoma obstructing the cystic duct. Case Report The patient, a thirty-nine year old black woman, was admitted to the Methodist Hospital to undergo elective cholecystectomy after nonvisualization of the gallbladder. She had had typical symptoms of gallbladder disease, including pain in the right upper abdominal quadrant and epigastrium with radiation to the back and infrascapular area, for approximately six years. The pain was unrelieved by antacids, frequently caused her to awaken at night, and was becoming increasingly severe. She denied having had jaundice. Physical examination demonstrated severe dextroscoliosis of the thorax but was otherwise noncontributory. Results of laboratory studies including liver function tests were likewise within normal limits. From the Cora and Webb Mading Department of Surgery. Baylor College of Medicine and the Methodist Hospital, Houston, Texas. * Present address and address for reprint requests: Texas Heart Institute, P.O. Box 20345, Houston, Texas 77025.
Volume 129, May 1975
Elective cholecystectomy was performed on April 5, 1973. The gallbladder was tense and distended but without palpable stones; however, a nodule was palpable in the cystic duct approximately 1 cm from its entrance into the common duct. The common duct and pancreas appeared normal. Routine cholecystectomy was carried out. White mucoid material was present in the distended gallbladder distal to a small nodule that appeared to arise in the valves of Hiester and that totally obstructed the cystic duct. From frozen section study of the nodule, a diagnosis of granular cell myoblastoma of the cystic duct was made. On further pathologic examination, the gallbladder measured 8 by 4 by 3 cm. The serosa was intact, and the wall of the gallbladder measured 3 mm in thickness. A thick layer of dense mucous material covered the gallbladder mucosa. In the terminal portion of the cystic duct, the wall appeared grossly thickened up to 5 mm in diameter. The thickening was eccentric. The mass involved an 8 mm portion of the terminal cystic duct. Microscopic examination demonstrated a granular cell tumor that involved the submucosa and muscularis of the cystic duct. The distal margin of the cystic duct was uninvolved. Microscopic section showed almost complete obliteration of the cystic duct. (Figures 1 and 2.1
Comment Granular cell myoblastoma is usually a benign tumor although occasional cases of malignancy have been reported [2-41. LiVolsi et al [I] have stated
that
some
malignant
variants
of granular
583
Reul, Rubio, and Berkman
Figure 1. Granular ceil tumor (lower half of photomicrograph) bulges into the lumen, causing marked stenosis of the cystic duct (hematoxyiin and eosin stain; original magnification X 45).
Figure 2. Tumor ceils in detail show characteristic granuiar cytoplasm and uniform, dark, ovokt nuclei (hematoxyiin and eosin stain; original magnification X 675).
cell tumors may actually represent multifocal lesions since granular cell tumors are frequently multicentric [4]. There is much controversy concerning the precursor cell of the tumor. Murray [5] has proposed that the granular cell tumor arises from a striated muscle cell, from which the term, “myoblastoma,” was derived [5]. However, others have suggested a neural origin since these cells resemble Schwann cells on electron microscopy [4-61. Granular cell myoblastoma of the biliary tract has been reported infrequently, there being only eleven cases in the literature [I]. All cases have occurred in females. Six lesions were found in the cystic duct, four in the common duct, and one in the common hepatic duct. Most patients had a preoperative diagnosis of cholelithiasis and nine of the eleven patients underwent surgery with no mortality or recurrence. In the remaining two patients the diagnosis was made at autopsy. Obstructive jaundice was found in two patients. Severe hepatic failure with hepatic coma resulted in one patient with obstruction of the common bile duct in whom the lesion was found at autopsy.
Summary
584
The first known case of true hydrops of the gallbladder that resulted from granular cell myoblastoma is reported. Granular cell tumors are benign multicentric tumors that should be treated by total excision so that free margins are assured. These tumors are rarely found in the biliary tract; however, they may cause complications of biliary tract obstruction.
References 1. LiVolsi VA, Perzin KH, Badder EM, Price JB, Porter M: Granular cell tumors of the bitiiry tract. Arch Pathol 95: 13, 1973. 2. Crawford ES, DeBakey ME: Granular cell myoblastoma: two unusual cases. Cancer 6: 786, 1953. 3. Ross RC, Miller TC, Foote FW: Malignant granular cell myoblastoma. Cancer5 112, 1952. 4. Svejda J. Horn V: A disseminated granular cell pseudotumor: so-called metastasizinggranularcell myoblastoma. J Pathol76: 343, 1958. 5. Murray MR: Cultural characteristics of three granular cell myoblastomas. Cancer 4: 857, 195 1. 6. Pearse AGE: The histogenesis of granular cell myoblastoma (7 granular cell perineural fibroblastoma). J Patio/ 62: 351, 1950.
The American Journal
of Suqery
