Ovarian hyperemia small cell carcinoma misdiagnosed as 1 case of granulomas

Ovarian hyper-blood calcium small cell carcinomatoskova? ryhypercalcemictype, SCCOHT) currently has no statistics in the countryThe age of onset is usually less than 40 years old, the median age of 24 years old, 2/3 of the patients combined with high blood calcium, the incidence is associated with SMARCA4 gene mutation, the recurrence rate is high and the prognosis is very poor, the vast majority of deaths within 2 years, surgery and platinum-based treatment is still the main treatment plan, but the effect is not accurateThe sCCOHT treatment of one case that was misdiagnosed as granulomoma in our hospital has been reported as follows1 medical record summarypatients 20 years old, due to "abdominal 20d" in the local hospital, CA125 205kU/L, MRI: pelvic abdominal cavity huge cystic tumor2018-01-23 line "Caesarean section , right attachment removal , left ovary biopsy , appendectomy , large mesh removal , pelvic lymph node sweeping"In the operative see: a small amount of blood-borne ascites, the right egg nest cyst swollen 20cm x 20cm, adhesion with the peritoneum, the preoperative part of the tumor has ruptured, turbid blood fluid outflowPostoperative disease test: ovarian adult high-scoring granulomoma, diagnosed as "ovary granulomoma I.Cperiod." 201802-08 to 2018-06-10 lines of yew alcohol and caplatinum chemotherapy 6 cycles, chemotherapy side-effect setherapy light, review CA125 and MRI prompt complete lying, after every 3 months to review CA125 and color super no abnormalityIn January 2019, abdominal pain accompanied constipation, the local hospital review MRI: ovarian cancer recurrence with pelvic peritoneal network transferFor further treatment into the Female Oncology Department of Chongqing University Affiliated Oncology HospitalThe first tide 13 years old, 3 to 4d/28d, the last menstruation 2019-01-01, unmarried uneducated, past history, personal history, family history no specialCheck body: right lower abdominal and 10cm size cystic activity package, the boundary is not clear, no pressure pain, rectal mucous membrane smoothTumor markers: CA125107.40kU/L, CA724 16.18 kU/L, CA199 10.39 kU/L, squamous cancer antigen (SCC) 1.20 mg/L, cancer embryo antigen (CEA) 0.50? G/L, meta-fetal protein (AFP) 2.50 ?g/L, neuron-specific oleolase (NSE) 4.17 ?g/L, human fluff membrane gonadotropin (hCG) 0.71?U/L, where CA125 and CA724 lElectrolyte: Blood calcium 2.99mmol/L, blood sodium, blood potassium normalThe whole body shallow lymph nodes, color super, chest CT, gastroscope, colonoscopy normal Basin abdominal MRI (Figure 1): basin abdominal cavity multiple cystic lump sympacity, the boundary is not clear, range 11.3cm x 10.0cm, flat sweep signal is not uniform, enhance the scanning real composition and the bladder wall significantly strengthened, the pelvic abdominal cavity a large amount of fluid, intestinal membrane and mesh thickening is cake-like PETCT: after the peritoneum, abdominal pelvic cavity multiple lumps, the boundary is not clear, the larger 11.7 cm x 12.9 cm, metabolism increased 2019-01-29 lines of caesarean section, see: blood-borne ascites 0.6L, top, liver, spleen, stomach, and abnormalities, pelvic cystsolids (real-based) 18 cm x 15cm, tumor surface see 3cm break, surface blood seepage, rectal and b-shaped colon wall scattered in the nipple-like lesions several 0.5 to 1.0cm, uterine size is normal, the back wall is invaded, and pelvic swollen Can not be separated, left fallopian tube umbrella side see nipple-like swelling 2cm x 2cm, left ovary cyst actual increase 5cm x 4cm, with the left pelvic wall and pelvic swelling dense adhesion, bladder reverse peritoneal nipple-like disease The stove 3cm x 3 cm, the right attachment is missing, the pelvic lymph nodes are not swollen and swollen, the lymph nodes next to the abdominal aorta are enlarged to be cystic 8cm x 6cm, the upper bound sands reach the top, and the duodenum is dense and adhesion No cancer cells were detected in the abdominal water Intraoperative freezing considers a tendency to interstitial cell tumors, cells significantly abnormal, reproductive cell source tumors can not be excluded Line tumor reduction (whole uterus , left attachment , right peritoneal swelling , and cosyumal surface swelling to remove), separation of band colon and rectal surface swelling, consider the abdominal aorta side lymph nodes wrapped around the abdominal aorta, lower cavity veins, intestinal membrane artery and duodenum starting part, can not be completely excision, after the procedure to put chemotherapy, to the silver clip marked lymph nodes below the boundary Surgical tumor reduction is not complete, residual lesions are located in the abdominal aorta next to the lymph nodes 8cm x 6cm, rectal surface lesions 1cm x 1cm Postoperative medical examination (Figure 2): hypercalcemia type small cell carcinoma, neuroaggression (-), tumor thrombosis (-) Immune grouping: CK-pan scattered in ( , , CK5/6), CK7 individual ( , , CK19 , CK20 , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , ,), CD56, WT-1, CD99 (?), alpha-inhibin (-), Melan A (A103) (-), OCT-4 (-), ki-67 (-) 60%, Cal retinin ( , , CAM5.2), D2-40 , TLE1 , Vimentin , PLAP Suspicious ( , , , CD34 Vasculars , ER ( -), PR (-), Her - 2 (-), MOC31 (-), Fli-1 (-), CEA (-), Desmin (-), NSE (-), Nestin (-), TTF1 (-), CD117 (-), CD10 Scatter in , INI-1 ( , , SOX10) ( -), S-100 (-) After surgery, the 6th day of exhaust defecation, restore the fluid diet On the 10th day after surgery, sudden abdominal pain, the drainage fluid is bright red about 0.4L, color super-show "the peritoneum after 10cm x 6cm cystic echo, abdominal fluid", consider tumor rupture bleeding, the proposed blood vessel intervention embolism to stop bleeding, and then chemotherapy and radiotherapy, the family refused to step into treatment, 1 month after discharge dying Combined with the morphological characteristics of postoperative medical examination, 2 discussed 2.1 clinical manifestations and auxiliary examination SCCOHT is a rare ovarian malignant tumor age 9 to 43 years old (average age 24 years), often for the monolith ovary, tumor size 6 to 26 cm (average 15cm) Clinical manifestations lack of specificity, can have abdominal pain, nausea, vomiting, wasting or menstrual disorders Imaging table is now cystic real swollen, the real main, compared with other types of ovarian cancer without obvious characteristics It has been reported that 62% of cases are associated with hypercalcemia, which is associated with tumor self-secreting parathyroid hormone, which causes calcium in the blood to rise in the bone and kidneys However, only a few cases showed clinical manifestations of high blood calcium, such as abdominal pain, thirst and fatigue Blood calcium levels can be used to assess efficacy and prognosis Secondary cancer syndrome systoses are hyponatremia, hypoglycemia, anti-diuretic hormone secretion, and muscle weakness Serum CA125 is 25 to 691kU/L (average 176kU/L), CA125 level is not associated with tumor load, metastases are similar to epithelial source ovarian cancer The patient in this case is 19 years old, clinical manifestations of abdominal pain and constipation, no specific symptoms, tumor is cystic, located in the right ovary, size 21cm, metastasis is located in the attachment, peritoneal, rectal surface, pelvic abdominal lymph nodes, tumor body prone to necrosis and rupture bleeding, both surgical probes are spontaneous rupture bleeding, the 10th day after surgery the main aortic hemorrhage At the time of initial treatment, CA125 was 205kU/L, reduced to normal after postoperative chemotherapy, 107kU/L after relapse, the elevation was not obvious, CA724 was slightly elevated, SCC, CEA, AFP, NSE, hCG and so on normal Preoperative blood calcium is 2.99 mmol/L, higher than normal, after surgery every 2d review of blood calcium fractions of 1.54, 1.87, 1.95, 2.17 mmol/L, are back to normal, may be related to tumor load reduction 2.2 histopathology SCCOHT tissue occurrence and cell origin is unknown, there are literature sedatives based on immunohistamytization and electromirror is considered to be a type of epithelial ovarian cancer, but its epithelial origin has not been confirmed There are also the theories of the source of reproductive cells, interstitial synods, and even neuroendocrine differentiation have been put forward Tumors to small cells diffuse flaky, nest-like, beam-like arrangement, cell size consistent, round or egg circle, cell pulp less, nuclear kernel obvious, nuclear division like more common, accompanied by a large number of necrosis and bleeding, some cases are follicles-like growth, follicle cavity contains acidophilic or alkaline liquid, a few cases contain cytoplasm rich crossfied fibroid-like large cells Immune phenotypes are characterized by expression epithelial markers (CK series, EMA) and neuroendocrine markers (CgA, Syn, CD56), and most cases also detect positive expressions of WT-1 and CD99, while signs of reproductive cell tumors, prosyzoa, and soft tissue tumors are all negative expressions SMARCA4-based mutation is a characteristic molecular genetic change of SCCOHT, with more than 69% of cases reported to have SMARCA4 gene mutations, and a few cases with SMARCB1 mutations This case of patients at the initial treatment of the external hospital misdiagnosed as a granule cell tumor, my hospital before the borrowing of the external hospital paraffin block pathology consultation also consider edgout cell tumor, my hospital in the freezing is still diagnosed unknown, tendentious isococell tumor, cell significant lysome, reproductive cell source tumor can not be excepted, abdominal water did not see cancer cells Until combined with the morphological characteristics of postoperative medical examination, immune groupCK series, EMA, CgA, Syn, CD56, WT-1, CD99 are positive expression, and the bed has high blood calcium, the final diagnosis, it can be seen that SCCOHT diagnosis is difficult 2.3 treatment SCCOHT prognosis is extremely poor, the vast majority of deaths within 2 years, surgery and platinum-based chemotherapy is still the main treatment, but the effect is not accurate, the scope of surgery, chemotherapy programs and radiotherapy status is still controversial Young and others reviewed 150 cases of SCCOHT, of which I.A patients had a better prognosis, a survival period of 1 to 13 years (average 5.7 years), a 5-year survival rate of 6% later than that of I.A patients, almost all deaths within 2 years, and only 1 case of period II.B after supplementary high-intensity chemotherapy and radiotherapy survival of 7 years Phase I.A., age greater than 30 years old, preoperative blood calcium normal, tumor less than 10cm, pathological appearance of large cell composition suggests a better prognosis There has also been literature reporting that tumor size is not related to prognosis, and that obvious lying-place symptoms contribute to early detection of tumors, with better prognosis Gamwell and other retrospective analysis of 17 cases of I.A patients, recommended curative surgery, postoperative supplementary chemotherapy and radiotherapy, because the disease is common in adolescents, and intraoperative freezing is often not diagnosed, can first routinely retain reproductive function surgery, postoperative medical examination and then supplementtheary surgery There are also reports of attempts to preserve reproductive function in i.A-phase infant son-in-law surgery, but on a case-by-case basis, and with poor efficacy For patients who are later than I.A, endothelial surgery and postoperative chemotherapy are the main treatment options, and new complementary chemotherapy is possible in cases where tumor reduction is expected Chemotherapy program recommends platinum-based combined chemotherapy, can refer to the VPCBAE program: Changchun new alkali 6mg/m2 plus cisplatin 90mg/m2D1, cyclophosphamide 1g/m2 and bolemycin 15U/m2D2, dojoobi star 60mg/m22, cycle 21 to 28 d 15-16 Nelsen et al reported three cases of combined pelvic radiotherapy, one of which survived for 5 years Radiotherapy can prolong the survival, but the premise is surgery to completely reduce the tumor, the efficacy of cases later than I.A or relapse is not satisfied, and pelvic radiotherapy is prone to radioactive enteritis, intestinal obstruction, intestinal perforation and other complications For SMARCA4 mutant gene carriers, it is necessary to closely follow blood calcium, CA125 and color super, may also consider post-birth prophylactic ovary excision Figure 1 basin abdominal MRI 2 postoperative pathology specimens and HE dyes (x100 immunohistomyded CK series, EMA, CgA, Syn, CD56, WT-1, CD99 amimall expression, and clinically high blood calcium, and was finally diagnosed as SCCOHT 2.4 Lessons learned SCCOHT pathology is easily misdiagnosed as a source of reproductive cells or interstitial, relying solely on pathological diagnosis is difficult, although the incidence is rare, but the clinician is familiar with its clinical performance and the characteristics of auxiliary examination, so as not to misdiagnosis In this case, patients were misdiagnosed as granulomas, the scope of surgery is insufficient and postoperative complementary treatment may not be appropriate, need to learn lessons SCCOHT is a special point for adolescents, often one-sided ovaries, tumors larger average 15cm, Imaging performance is cystic swollen, substance-based, can have high blood calcium, CA125 level and tumor load is not related, and CA125 rise is not obvious, tumor often accompanied by necrosis and bleeding, cell volume is small, such as pathological diagnosis for reproductive cells, interstitial, small cell neuroendocrine cancer or non-differentiated, need to communicate with the pathologist, if necessary, high-level hospital consultation It is recommended that no matter whether the phase dissection of the morning or evening to carry out the endothellan surgery, do not retain reproductive function, such as the misdiagnosis of the procedure for reproductive cells or the source of interstitial prosthesis, has been carried out to retain reproductive function surgery, after postoperative pathology diagnosis, it is recommended to supplement secondary surgery After the operation to take comprehensive treatment, timely adequate treatment of VPCBAE program chemotherapy, conditional hospitals may consider pelvic radiotherapy Although SCCOHT prognosis is very poor, but I.A., age greater than 30 years old, preoperative blood calcium normal, tumor less than 10 cm, pathological appearance of large cell components patients after comprehensive treatment is still expected to obtain a longer survival period At present, the scope of hand surgery, chemotherapy program and the status of radiotherapy need to be explored and studied references