Fig. 1(A) Abdominal CT scan of case no. 4. A low-attenuated, para-aortic retroperitoneal mass is observed. (B) Chest computed tomography (CT) scan of case no. 4. Multiple well-defined, uniformly thin-walled cysts are observed diffusely distributed throughout both lungs. (C) Gross examination of multiple dissections revealing a soft, well-circumscribed, lobulated, ivory mass in case no. 2. (D) Microscope images of the masses observed in cases nos. 1 and 4. These masses are composed of bland-looking spindle cells and show trabecular bundles with anastomosing patterns (case no. 1). (E) Nuclear morphology of cases nos. 2 and 3. Nuclear crowding is observed, along with mild to moderate nuclear atypism and conspicuous nucleoli. (F) Perivascular epithelioid E-LAM (PEE) cell proliferation detected at the intratumoral area of case no. 2. The PEE cells stain more strongly positive for human melanoma black-45 (HMB-45) than do spindle tumor cells. (inset) (G) Positive staining for HMB-45 in spindle tumor cells (case no. 2). (H) Diffuse and strong positive staining for smooth muscle actin in spindle tumor cells (case no. 2). (I) Positive staining for the progesterone receptor in the nuclei of the tumor cells (case no. 1).
Table 1.Clinicoradiologic data from patients with extrapulmonary lymphangioleiomyoma in Korea
Case No. |
Age (yr) |
Sex |
Location |
Size (number) |
Radiologic impression |
Other condition |
TS |
FUP (mo) |
1 |
47 |
F |
Parametrium |
6.5 (× 1) |
E-LAM |
P-LAM |
No |
95, alive, NER |
2 |
46 |
F |
Retroperitoneum (para-aortic) |
9 (× 1) |
Paraganglioma |
No |
No |
17, alive, NER |
3 |
46 |
F |
Right pelvic lymph node |
Up to 0.4 (× 3) |
No detection |
Sqcc in uterine cervix |
No |
19, alive, NER |
4 |
43 |
F |
Retroperitoneum (para-aortic) |
10 (× 1) |
Lymphoma |
P-LAM |
No |
1/3, alive, NER |
Kim et al. [7] |
21 |
F |
Pelvic cavity |
9.7 |
N/A |
P-LAM |
No |
6, died for P-LAM |
Han et al. [8] |
46 |
F |
Pelvic cavity |
5 |
N/A |
No |
N/A |
6, NER |
Table 2.Histopathologic information from patients with extrapulmonary lymphangioleiomyoma
Case No. |
Nuclear crowding |
Nucleoli |
Coarse chromatin |
Irregularity of nuclear membrane |
Cytoplasmic |
Perivascular epithelioid E-LAM cells |
Slit-like space |
Mitosis/Necrosis |
HMB-45/SMA |
1 |
– |
Inconspicuous |
– |
– |
– |
– |
+ |
–/– |
+/+ |
2 |
+ |
Conspicuous |
+ |
+ |
+ |
+ |
+ |
–/– |
+/+ |
3 |
+ |
Conspicuous |
+ |
+ |
+ |
– |
+ |
–/– |
+/+ |
4 |
– |
Inconspicuous |
– |
– |
– |
– |
+ |
–/– |
+/+ |