LETTER TO EDITOR
Year : 2010 | Volume
: 53 | Issue : 1 | Page : 173--175
Perinodular hydropic degeneration in leiomyoma: An alarming histology
Kusum D Jashnani, Sangeeta Kini, Gaurav Dhamija
Department of Pathology, BYL Nair Hospital & TNMC, Mumbai-400 008, India
Flat No. A-802, Keshav-Kunj III, Sector 14, Plot 19, Sanpada, Navi Mumbai - 400 705
|How to cite this article:|
Jashnani KD, Kini S, Dhamija G. Perinodular hydropic degeneration in leiomyoma: An alarming histology.Indian J Pathol Microbiol 2010;53:173-175
|How to cite this URL:|
Jashnani KD, Kini S, Dhamija G. Perinodular hydropic degeneration in leiomyoma: An alarming histology. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jan 26 ];53:173-175
Available from: https://www.ijpmonline.org/text.asp?2010/53/1/173/59223
Uterine leiomyomas have a spectrum of histology and growth patterns following various degenerative changes. This often causes diagnostic difficulty. It is essential to recognize and differentiate conventional leiomyomas from various other alarming variants as some of these are fraught with diagnostic difficulty histologically when located extra uterine and can lead to overt management. This was observed in six cases described in literature.  We describe three such cases of perinodular hydropic degeneration in leiomyoma with two being extra uterine, thus imposing diagnostic difficulty clinically as well on histology.
Case 1 : A 50-year-old woman presented with distension and pain in abdomen of one year duration. On local examination, a non-tender minimally movable abdominal palpable mass attached to rectus abdominus was noted. A Computed tomography (CT) abdomen revealed a 7.2 cm x 2.7 cm well defined lobulated intensely enhancing lesion in the infraumbilical region arising from posterior rectus sheath with foci of calcification. The mass was seen in continuity with uterine fundus and the right ovary could not be visualized. Hence a diagnosis of right ovarian dermoid was rendered [Figure 1]. Other routine investigations including tumor markers (CEA, CA-125) were within normal limits. Intraoperatively, the mass was seen adhered to the posterior rectus muscle, lateral pelvic wall and rectum; a malignant neoplasm was suggested on clinical grounds.
Resected specimen of the abdominal mass received was an irregular, nodular, partly capsulated, solid mass measuring 7 cm x 7 cm x 4 cm. Cut surface was tan-white lobulated with multiple gritty lumina resembling calcified vessel wall. Separately sent atrophic uterus with cervix measured 4cm x 4 cm x 2cm and had a pedicle at the fundus, the cut surface of which had similar appearance that of the abdominal mass [Figure 2]. Bilateral tubes and ovaries were unremarkable.
Microscopic examination of the abdominal mass and uterine fundal pedicle had similar histology. It showed tumor with nodules of variable size, shape and cellularity separated by hydropic and hyaline degeneration. Cellular nodules were composed of fascicles and bundles of spindle smooth cells. Other nodules had large hyalinized thick walled blood vessels with calcification suggestive of Monckeberg's sclerosis [Figure 3]. Some nodules were vascular, composed of small sized vascular channels mimicking a vascular neoplasm [Figure 4]. Foci of severe degree of nuclear atypia and multinucleate giant cells were noted, although there was absence of abnormal mitosis and coagulative necrosis [Figure 4]. On extensive search, focal features of conventional leiomyoma helped in giving a diagnosis of perinodular hydropic and hyaline degeneration in leiomyoma. Special stains performed confirmed smooth muscle origin. Supplementary immunohistochemistry (IHC) had been done which showed strong positivity for h-caldesmon and focal positivity for SMA, thus confirming smooth muscle origin of the lesion [Figure 4].
Case 2 : A 40-year-old woman presented with pain in right iliac fossa and oligomenorrhoea of four to five months duration. Per vaginal examination revealed a freely movable mass in the right fornix, a hypo echoic well-defined lesion measuring 4.2 cm x 3.2 cm x 3.1 cm with linear shadowing in right adnexae on ultrasonography abdomen suggesting possibility of a broad ligament fibroid.
Resected specimen of total hysterectomy showed presence of a broad ligament mass measuring 4 cm x 3 cm x 2cm with bosselated external surface and a cut surface similar to case number 1 except for calcific foci.
Case 3: A 33-year-old woman Gravida0 Para0 Aborted1 investigated for primary infertility came with complaints of vague abdominal pain. A per abdomen examination revealed a 22-week uterus with a mobile mass confirmed on ultrasonography abdomen as presence of multiple fibroids in the uterus. Resected specimen of the hysterectomy showed presence of a large fundal fibroid measuring 16 cm x 11 cm x 4 cm in addition to multiple smaller fibroids. The cut surface was similar to case 1 except for calcific foci. Histology of both case 2 and case 3 showed features of perinodular hyaline and hydropic degeneration in leiomyoma without calcification of the thick walled blood vessels. Follow-up of all the three cases suggest a benign nature of the lesion.
Leiomyoma, the most common benign smooth muscle tumor of uterus often presents with various degenerative changes such as hyaline, cystic, calcification, and hydropic types of degeneration. Histomorphology of leiomyoma with the degenerative changes occurring as focal with in recognizable leiomyoma is not difficult to diagnose. However, when present in abundance, distorting the architectural pattern of leiomyoma and extending outside the uterus can lead to erroneous diagnosis. This is true particularly with variable amounts of hydropic and hyaline degeneration occurring in conjunction and leading to possibilities of myxoid leiomyosarcoma, vascular tumors such as angiofibromas or angiomyxoma or endometrial sarcoma. ,
The variants of leiomyoma with variable amount of hydropic and hyaline degeneration are diffuse leiomyomatosis, dissecting leiomyoma, cotyledenoid and perinodular hydropic degeneration.  Clement et al.  were the first one to describe in their series of 10 cases of uterine leiomyomas with hydropic and hyaline degeneration creating diagnostic problems.
Coad et al.  were the first to describe perinodular hydropic degeneration of leiomyoma in extrauterine location as was observed in the present series of two of the three cases described. On gross, this type of leiomyoma has vague multiple variably sized pink-tan to white-gray nodules as was observed in all three cases described. However, in addition, in one our cases we found prominent calcified vascular lumina.
Literature shows, on histology, the perinodular type has a nodular pattern of cellular leiomyomatous appearance with variable hyaline and hydropic stroma which in turn is at times richly vascularized and can resemble a hemangioma. The hypothesis is that this stroma gives a tan appearance to the tumor on gross morphology.
Special stains such as trichrome for muscle is sufficient for making a diagnosis. However, when clinically presenting at extra uterine location and to confirm smooth muscle origin in the absence of recognizable leiomyomatous component on histology, a battery of immunohistochemical markers can be done for both smooth muscle and vessels to differentiate the origin. 
In conclusion, perinodular hydropic degeneration in leiomyoma is a rare appearance of leiomyoma. Awareness of these changes in leiomyoma, particularly when occurring in extra uterine location is essential and residual foci of recognizable leiomyoma should be looked for to prevent making an erroneous diagnosis of vascular tumor and overt management.
We thank Dr Santosh Menon, Consultant,Tata Memorial hospital, for carrying out immunohistochemistry.
|1||Ceyhen K, Simsir C, Dolen I, Calyskan E, Umudum H. Multinodular hydropic leiomyoma of the uterus with perinodular hydropic degeneration and extrauterine extension. Pathol Int 2002;52:540-3.|
|2||Clement PB, Young RH, Scully RE. Diffuse, perinodular, and other patterns of hydropic degeneration within and adjacent to uterine leiomyomas. Problems in differential diagnosis. Am J Surg Pathol 1992;16:26-32.|
|3||Coad JE, Sulaiman RA, Das K, Staley N. Perinodular hydropic degeneration of a uterine leiomyoma: A diagnostic challenge. Hum Pathol 1997;28:249-51.|
|4||Maimoon S, Wilkinson A, Mahore S, Bothale K, Patrikar A. Cotelydonoid leiomyoma of the uterus. Indian J Pathol Micro 2006;49:289-91.|
|5||Fukunaga M, Ushigome S. Dissecting leiomyoma of the uterus with extrauterine extension. Histopathology 1998;32:160-4.|