Author Sex, age Origin LNM Treatment Adjuvant treatment Follow-up
Cui et al. [19] Male, 47 yrs Kidney Multiple  paraortal LNM Nephrectomy and LND No No evidence of disease, 2 yrs FU
Liu  et al. [20] Female , 33 yrs Uterus Multiple  LNM Neoadjuvant chemotherapy (type unknown), debulking Chemotherapy
(type unknown),
No evidence of disease, 8 months FU
Bonis  et al. [21] Female, 39 yrs Pelvis Multiple  paraortal LNM Tumor excision left pelvic and  paraortal LND No No evidence of disease, 1 yr FU
Huang  et al. [22] Female, 78 yrs Kidney Regional LNM Radical nephrectomy No Died of disseminated disease (lungs and bones) 5 months postoperatively
Chen  et al. [23] Female, 16 yrs Pelvis Enlarged pelvic, mesocolon, iliac  LNM Debulking and pelvic floor LND Chemotherapy
(type unknown),
Recurrence 2 months after debulking
Harris  et al. [24] Male, 87 yrs Knee Recurrence -1/8 Inguinal LNM and pulmonary metastases Primary debulking Groin LND Alive with disease 40 months FU
Bonetti  et al. [25] 1.  Female, 28 yrs Serosa ileum Mesenteric  LNM Tumour resection No Died 28 months after surgery because of liver insufficiency due to hepatic metastases
2.  Female, 19 yrs Uterus 1 month after surgery pelvic LNM Hysterectomy – 1 month later pelvic LND and inguinal LND Adriamycin, ifosfamide, radiotherapy 10 months after chemotherapy: local recurrence, lung and bone metastases, died 18 months after surgery
Daraï   et al. [10] Female, 18 yrs Corpus uteri Recurrence - Pelvic  LNM Excision   Alive with disease 24 months
Ryan  et al. [26] Female, 15 yrs Rectum Mesenteric LNM Low anterior resection Doxorubicin, ifosfamide No evidence of disease 9 months FU
Liang  et al. [11] Female, 59 yrs Corpus uteri 3/13 pelvic and para-aortal LNM: proliferation of benign smooth muscle bundles (LLM) Radical hysterectomy, BSO pelvic LND, paraortal LND, omentectomy, appendectomy None No information
Calder  et al. [27]   Unknown primary Cervical LNM Excision No 5 yrs FU scalp noduleà excision. Hereafter no information.
Vang  et al. [12] 1.  Female, 49 yrs Corpus uteri Pelvic LLM Radical hysterectomy, BSO No No evidence of disease, 4.5 yrs FU
2.  Female, 58 yrs Corpus uteri and  endometrial cancer Pelvic LLM Radical hysterectomy, BSO No Lost to follow-up
Jeon  et al. [9] Female, 9 yrs Corpus uteri 1 pelvic, paraortal and precaval  LNM 2 cycles neo-adjuvant chemotherapy with vincristine/ifosfamide/ doxorubincin (VID), hysterectomy and pelvic LND 6x VID regimen, radiotherapy 45 Gray No evidence of disease 1.5 yrs FU, at 2 yrs FU the patient developed acute lymphoblastic leukaemia, treated and in the maintenance phase [28]
LNM: lymph node metastases
LND: lymph node dissection
FU: follow-up
BSO: bilateral salpingoophorectomy
LLM: lymphangioleiomyomatosis = pecomatosis
Table 1: Systematic literature search involving all PEComa cases with lymphatic metastases.
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