DIVU SINHRONU NEATKARĪGU PRIMĀRU ĻAUNDABĪGU AUDZĒJU MANIFESTĀCIJA: METASTĀTISKA VULVAS PLAKANŠŪNU KARCINOMA UN MUTIFOKĀLA UROTELIĀLA KARCINOMA
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Date
2021
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
IEVADS.
Multiplu primāru ļaundabīgu audzēju incidence ir zema, svārstoties 0.3-4.3%. Līdz šim nav ziņots par klīnisku gadījumu, kad sinhroni attīstījusies metastātiska vulvas plakanšūnu karcinoma un multifokāla uroteliāla karcinoma. Turklāt vulvas vēzis sastopams vien 3-5% no visiem sieviešu ģenitālā trakta ļaundabīgajiem audzējiem, un tā metastāzes ādā ir uzskatāmas par ārkārtīgi retu parādību.
GADĪJUMA APRAKSTS.
Septiņdesmit piecus gadus veca sieviete vērsās pie onkoginekologa ar sūdzībām par strauji augošu masu vulvā. Objektīvās izmeklēšanas laikā tika konstatēts kreisās puses lielās kaunuma lūpas veidojums, eksofīts ādas veidojums kaunuma paugura rajonā un palielināti kreisās puses cirkšņa limfmezgli. Vulvas veidojuma biopsijā tika apstiprināta vidēji diferencēta plakanšūnu karcinoma.
Vēdera dobuma un iegurņa datortomogrāfijā tika aprakstīts vulvas audzējs (8x2.3 cm) un specifiska kreisās puses cirkšņa limfadenopātija. Negaidīti radiologs atklāja arī veidojumu labās nieres augšpolā (7x4.2cm), kā arī invazīvu veidojumu urīnpūslī (2.8x3.4cm) ar ieaugšanu labajā urīnvadā. Attālas metastāzes netika atrastas.
Multidisciplināra komanda rekomendēja pacientei sarežģītu divu etapu ķirurģisku ārstēšanu. Pirmkārt, tika veikta kreisās puses iegurņa limfmezglu dissekcija un to histoloģiska izmeklēšana attālu limfogēnu metastāžu izvērtēšanai. Tam sekoja laparatomiska labās puses nefroureterektomija ar daļēju cistektomiju un totāla histerektomija ar bilaterālu adneksektomiju. Operācijas otrais etaps ietvēra ādas veidojuma ekscīziju, kreisās puses hemivulvektomiju un bilaterālu ingvinofemorālu limfadenektomiju. Tika veikta vulvas rekonstrukcija ar V-Y lēveri.
Pēcoperācijas histoloģiskā izmeklēšana apstiprināja divus neatkarīgus ļaundabīgus audzējus – vidēji diferencētu vulvas plakanšūnu karcinomu ar metastāzēm ādā un kreisā cirkšņa limfmezglos, kā arī zemu diferencētu uroteliālu karcinomu nierē, urīnvadā un urīnpūslī.
Pacientei tika nozīmēts staru terapijas kurss 48 Gy apmērā, 2 Gy frakcijās. Novērošanas laikā gadu pēc terapijas netika atklāts slimības recidīvs.
SECINĀJUMI.
Rūpīga onkoloģiskā pacienta izmeklēšana ļauj noteikt ne tikai konkrētā audzēja izplatību un klīnisko stadiju, bet arī atklāt slēptus ļaundabīgus veidojumus. Sinhronu neatkarīgu audzēju ārstēšana ir profesionāls izaicinājums, jo nepieciešama rūpīga terapijas taktikas izplānošana un pielāgošana pacientam, lai sasniegtu labāko iespējamo rezultātu. Mūsdienu radikālās ķirurģijas metodes sniedz iespēju uzlabot pacientu dzīvildzi un dzīves kvalitāti.
INTRODUCTION. The incidence of multiple primary cancers is 0.3-4.3%. The cases of synchronous metastatic vulvar squamous cell carcinoma (SCC) and urothelial carcinoma (UC) have not been reported in the past. Furthermore, vulvar cancer represents only 3-5% of female genital tract malignancies, and cutaneous metastases from it are extremely rare - reported in only a few cases. CASE DESCRIPTION. A 75-year-old woman presented with a rapidly growing mass of the vulva. Physical examination revealed a tumor on the left labia majora, an exophyte formation of the skin of mons pubis and enlarged left inguinal lymph nodes (LN). Histological analysis showed moderately differentiated SCC. A CT scan confirmed the vulvar tumor(8x2.3cm), specific lymphadenopathy in the left groin, as well as unexpectedly found a mass at the upper pole of right kidney(7x4.2cm) and a tumor of the bladder(2.8x3.4cm) invading the right ureteral ostium. Distant metastases were absent. Multidisciplinary team recommended a complex two-step surgery. Firstly, left pelvic LN dissection was done followed by frozen section histology. After that laparotomic nephroureterectomy with partial cystectomy and total hysterectomy with bilateral adnexectomy were performed. The second step included excision of skin lesion, left hemivulvectomy with reconstruction and bilateral inguinofemoral lymphadenectomy. Pathological evaluation showed two independent malignancies - moderately differentiated SCC of the vulva with metastasis in the skin and inguinofemoral LN and poorly differentiated UC of the kidney, ureter and bladder. Patient received radiation therapy with 48 Gy in 2 Gy fractions. Woman was disease-free after 12 months follow-up. CONCLUSIONS. Thorough investigation of the oncological patient may reveal not only the dissemination of tumor, but also an occult malignancy. Treatment of double malignancies is a professional challenge that requires careful planning and multidisciplinary approach to find the best option for the patient. Modern radical surgery could prolong survival and improve the quality of life.
INTRODUCTION. The incidence of multiple primary cancers is 0.3-4.3%. The cases of synchronous metastatic vulvar squamous cell carcinoma (SCC) and urothelial carcinoma (UC) have not been reported in the past. Furthermore, vulvar cancer represents only 3-5% of female genital tract malignancies, and cutaneous metastases from it are extremely rare - reported in only a few cases. CASE DESCRIPTION. A 75-year-old woman presented with a rapidly growing mass of the vulva. Physical examination revealed a tumor on the left labia majora, an exophyte formation of the skin of mons pubis and enlarged left inguinal lymph nodes (LN). Histological analysis showed moderately differentiated SCC. A CT scan confirmed the vulvar tumor(8x2.3cm), specific lymphadenopathy in the left groin, as well as unexpectedly found a mass at the upper pole of right kidney(7x4.2cm) and a tumor of the bladder(2.8x3.4cm) invading the right ureteral ostium. Distant metastases were absent. Multidisciplinary team recommended a complex two-step surgery. Firstly, left pelvic LN dissection was done followed by frozen section histology. After that laparotomic nephroureterectomy with partial cystectomy and total hysterectomy with bilateral adnexectomy were performed. The second step included excision of skin lesion, left hemivulvectomy with reconstruction and bilateral inguinofemoral lymphadenectomy. Pathological evaluation showed two independent malignancies - moderately differentiated SCC of the vulva with metastasis in the skin and inguinofemoral LN and poorly differentiated UC of the kidney, ureter and bladder. Patient received radiation therapy with 48 Gy in 2 Gy fractions. Woman was disease-free after 12 months follow-up. CONCLUSIONS. Thorough investigation of the oncological patient may reveal not only the dissemination of tumor, but also an occult malignancy. Treatment of double malignancies is a professional challenge that requires careful planning and multidisciplinary approach to find the best option for the patient. Modern radical surgery could prolong survival and improve the quality of life.
Description
Pediatrija
Pediatrics
Veselības aprūpe
Health Care
Pediatrics
Veselības aprūpe
Health Care
Keywords
vulvas plakanšūnu karcinoma, uroteliāla karcinoma, vulvar squamous cell carcinoma, urothelial carcinoma