Plasmacytoid cystic adenocarcinoma of the ovary
Guo××, female, 45 years old, farmer, was admitted to the hospital on May 6, 2001 due to “abdominal distension and pain for six months, pelvic mass found on physical examination”.
The patient had a regular menstrual cycle of 4 to 6/30 days, with moderate volume and no dysmenorrhea, and her LMP: April 28, 2001. 2 years ago, she visited a local hospital, where an ultrasound detected a 4 cm x 3 cm x 3 cm mass in her right ovary. Six months ago, she developed right upper abdominal distension and pain without any obvious cause, which was diagnosed locally as “cholecystitis”. Since the onset of the disease, he was mentally fine, slept well, ate poorly, and lost about 6 kg in the past 4 months. She was admitted to the hospital for examination: T: 36.5℃, P: 78 beats/min, R: 18 beats/min, BP: 130/80 mmHg. Middle-aged female, normal development, general nutrition, clear mental health, mental health, autonomic posture, and cooperation in physical examination. There was no yellowish staining of the skin and mucous membranes, no swelling, and enlarged lymph nodes of about 1.5 cm in diameter in the left groin and about 2 cm in diameter in the right groin. There was no abnormality on the head and face. The neck was soft, the veins were not angry, the trachea was centered, the thyroid glands were symmetrical, and there were no enlarged hard nodes. The thorax was symmetrical, the breasts were well developed, and the heart and lungs were not abnormal. The abdomen is froggy, soft, not very tense, the liver and spleen are not reached under the ribs, the lower abdomen can be palpated mass, cystic solid, surface is not flat, poor activity, positive mobile turbid sounds, normal bowel sounds. There was no percussion pain in both kidney areas. The spine was physiologically curved, the limbs moved freely, physiological reflexes existed, and pathological reflexes were not elicited. Song Kun, Department of Gynecology, Qilu Hospital, Shandong University
Gynecological examination: vulva is married and delivered type, vagina is patent, leukorrhea is not abundant, cervix is smooth, size and shape are normal, uterine body is flat, slightly enlarged, clear boundary, tough, good activity, no pressure pain, left adnexal mass is about 4 cm×2 cm×2 cm in size, right adnexal mass is about 4 cm×3 cm×2 cm in size, irregular shape, unclear boundary, hard, surface is not flat, poor activity, tenderness Positive, hard nodules could be palpated in the rectal recess of the uterus, and the size and shape were unsatisfactory on palpation.
Auxiliary examinations: 1. Blood count: WBC:11.4×109/L, RBC:3.55×1012/L, Hb:90g/L, Plt:233×109/L; 2. Liver function: alkaline phosphatase 106 u/l, the rest normal; 3. Pelvic and abdominal ultrasound: uterus anteriorly positioned, 9.0 cm×6.0 cm×5.1 cm in size, regular shape. The left adnexal area showed an irregular solid mass of 3.9 cm×2.4 cm in size, and the right adnexal area showed an irregular solid mass of 4.9 cm×2.2 cm in size. The right hepatic peritoneum was uneven, the intrahepatic light spot was still homogeneous, and both kidneys were normal.4. Tumor markers: CA125: >600 u/ml, SA: 910 µg/ml, TSGF: 75 u/ml.5. CT findings: combined with the medical history, they were consistent with ovarian cancer combined with peritoneal as well as retroperitoneal lymph node metastasis and ascites.
Preliminary diagnosis: 1, advanced ovarian cancer 2, ascites
Treatment: After admission to the hospital, we actively improved all the auxiliary examinations and performed a caesarean section under general anesthesia on May 10. About 1,500 ml of peritoneal fluid was extracted during the operation, which was yellowish in color, and the cancer cells were detected by cytological examination. Multiple nodular metastases were seen on the surface of the greater omentum, mesentery and small intestine, ranging in size from 1 cm to 5 cm in diameter. 5 cm x 5 cm x 4 cm metastases were seen in the hepatic flexure of the colon, 4 cm x 4 cm x 3 cm ileocecal mass, 5 cm x 6 cm x 5 cm left ovarian tumor with cauliflower shape, 6 cm x 5 cm x 5 cm right ovarian tumor with cystic solidity and cauliflower shape. The right ovarian tumor was 6 cm×5 cm×5 cm in size, cystic solid and cauliflower-shaped, and the bilateral fallopian tubes showed corn-like implantation foci. The tumor cytoreductive surgery (total hysterectomy + double adnexa + large omentum resection, partial small bowel resection, ascending colon + transverse colon resection and intestinal anastomosis, tumor lesion excision, pelvic and para-aortic lymph node dissection) was performed, and the residual tumor lesion was <2 cm in diameter. Postoperative conventional pathology reported plasmacytic papillary cystic adenocarcinoma of the ovary (moderately differentiated) with retroperitoneal lymph nodes, large omental metastases, and extensive pelvic and abdominal metastases, FIGO stage IIIc. Postoperatively, 1 course of chemotherapy was administered using the PC regimen, and the patient was discharged after recovery. The patient was followed up regularly with monthly adherence to chemotherapy, and a total of 8 courses of chemotherapy with PC regimen were administered, with no evidence of recurrence at follow-up to date.
DISCUSSION
Ovarian tumors are a common type of gynecologic tumors that can develop at all ages. The incidence of ovarian malignancy accounts for 2.4% to 5.6% of common malignancies in women. Because of its anatomical location deep in the pelvic cavity, it is not easy to be detected and difficult to be diagnosed, and most of the malignant tumors are already in advanced stage when diagnosed. Ovarian tumors are broadly divided into tumors of epithelial origin, sex cord-mesenchymal tumors, germ cell tumors and metastatic tumors according to histological types. Among them, epithelial tumors are the most common ovarian tumors, accounting for about 50% of benign ovarian tumors and 85% to 90% of primary malignant tumors of the ovary. Epithelial tumors are most commonly seen in middle-aged and older women and rarely occur in prepubertal girls and infants.
Classification of epithelial ovarian tumors
1. plasmacytoma benign, junctional and malignant
2. mucinous tumors benign, junctional and malignant
3. Endometrioid tumors benign, junctional and malignant
4.clear cell tumor benign, junctional and malignant
5.Migratory cell tumor benign, junctional and malignant
6.Mixed epithelial tumors benign, junctional and malignant
7.Undifferentiated carcinoma
8.Cannot be classified and other epithelial tumors
Pathology of plasmacytoid ovarian cancer
Ovarian plasmacytoma is the most common ovarian epithelial tumor, accounting for 40% of ovarian tumors. Plasmacytoid malignant tumors account for 50% of ovarian cancer.
1. Gross findings: the volume can be large or small, with an average diameter of 10 cm to 15 cm. 1/3 to 1/2 are bilateral. The cystic surface is solid, with many brittle papillae and solid nodules. The contents of the capsule are watery or plasmacytic. About 28% of benign and malignant plasmacytic tumors may contain viscous mucus. Exophytic papillae, tumor papillae growing directly from the cortical surface of the ovary, are often bilateral and have extensive abdominal dissemination at the time of presentation. In plasmacytoma, abdominal dissemination is rapid and may be accompanied by large amount of ascites.
2. Microscopic view: The cancer papillae are not only branched but also bridged to each other. According to the differentiation of cancer cells, they can be divided into highly differentiated, moderately differentiated and poorly differentiated. The cancer cells can accumulate significantly, present papillary protrusions and infiltrate into the interstitium; or the cells are small, poorly differentiated, with deep-stained nuclei and splitting signs; those with poor differentiation have few or no papillary structures, few glandular-like structures, obvious heterogeneity, and serious invasion of interstitium and envelope. Sand granules are characteristic of this carcinoma, but not specific, also seen in benign plasmacytic cystadenoma.
Tumor staging (FIGO 1985)
Stage I Tumor limited to the ovary
Ⅰa Tumor limited to one ovary, no ascites, no tumor on the surface, intact envelope
Ⅰb Tumor limited to both ovaries, no ascites, no tumor on the surface, intact envelope
Ⅰc Stage Ⅰa or Ⅰb tumor, but tumor on the surface of one or both ovaries; or peritoneal rupture; or ascites containing malignant cells; or positive peritoneal washings
Stage II One or both ovarian tumors with intrapelvic spread
Ⅱa Spread and/or metastasis to uterus and/or fallopian tubes
Ⅱb Spread to other pelvic tissues
Stage IIc Stage IIa or IIb tumor but with tumor on the surface of one or both ovaries; or peritoneal rupture; or presence of ascites containing malignant cells; or positive peritoneal washings
Stage III One or both ovarian tumors with extra-pelvic peritoneal implants and/or positive retroperitoneal or inguinal Limba nodes. Liver surface metastasis is designated as stage III
IIIa Tumor limited to the true pelvis by visualization and negative lymph nodes, but histologically confirmed microscopic implantation on the peritoneal surface.
IIIb One or bilateral ovarian tumor with histologically confirmed peritoneal surface implantation, none of which exceeds 2 cm in diameter, and negative lymph nodes
IIIc Abdominal implantation >2 cm in diameter and/or positive retroperitoneal or inguinal lymph nodes
Stage IV One or both ovarian tumors have distant metastases. Stage IV if there are cancer cells in the pleural fluid and stage IV if there is metastasis in the liver parenchyma
Clinical manifestations
Ovarian epithelial carcinoma rarely occurs in prepuberty and is rare before the age of 40 years, after which the incidence rises sharply and then declines after the age of 60 years.
Abdominal mass is the most common symptom. When the abdominal mass is small in the early stage, it is not easily detected by the patient. When the mass is large or there is ascites, there may be a feeling of abdominal distension. When there are implantation metastases in the pelvis or abdominal cavity, or when the position changes so that the mass pulls the surrounding organs or has torsion, there can be abdominal pain symptoms. In this case, the patient came to the clinic with the main complaint of abdominal distension and pain.
Late stage ovarian cancer may have gastrointestinal symptoms such as low fever, loss of appetite, nausea, vomiting, constipation or diarrhea. Sometimes it is accompanied by pressure symptoms such as shortness of breath or frequent urination.
Ascites is a common sign, and many patients visit the doctor because of a series of symptoms produced by ascites. Pleural fluid is also occasionally seen.
Metastatic pathways
1. Direct implantation The most common site of ovarian epithelial cancer metastasis is the peritoneum, i.e. the peritoneum of the abdominal wall and the plasma membrane of the abdominal organs, including the diaphragm, omentum, small intestine collaterals, rectum, uterine rectal fossa, colon and the plasma membrane layer of the fallopian tubes and uterus. Therefore, most of the metastatic sites are on the surface of the organs exposed in the abdominal or pelvic cavity. Among the above metastatic sites, the utero-rectal fossa is the most common. Because of gravity, cancer cells are easily implanted in the lowest site. In this case, the extensive implantation foci in the pelvic and abdominal cavity were the result of direct implantation of cancer cells.
2. Spread with lymphatic drainage The lymphatic tract is also an important metastatic route. Cancer cells can travel along the ovarian vessels, from the ovarian lymphatic vessels upward to the parietal aortic lymph nodes; from the ovarian portal lymphatic vessels to the internal and external iliac lymph nodes, through the common iliac lymph nodes to the parietal aortic lymph nodes; along the round ligament into the external iliac and inguinal lymph nodes. In the present case, enlarged metastatic lymph nodes could be palpated in the groin bilaterally.
Although the uterus, fallopian tubes, and vagina are around the ovaries, metastases to these organs are mostly limited to the plasma layer, and metastases within the organ parenchyma are uncommon.
Hematogenous metastasis of ovarian cancer is rare and only seen in some advanced cases.
Treatment
The treatment of ovarian malignant tumors is mainly surgical, and then the need for chemotherapy or radiotherapy after surgery is decided according to clinical staging, histological category, and metastatic site, which in turn is decided according to dissection surgery.
1. Staging surgery Once the ovarian malignant tumor is suspected, the abdomen should be dissected as soon as possible. After opening the abdomen, ascites or peritoneal washings should be taken for examination to find out whether there are cancer cells; then the pelvic and abdominal cavity should be fully explored, including the diaphragm, liver, spleen, digestive tract, mesentery, wall peritoneum, internal genitalia, retroperitoneal lymph nodes, etc. Multi-point biopsy should be done for suspected lesions and parts prone to metastasis. According to the exploratory node country, the tumor stage and the scope of surgery will be decided. For stage Ⅰa and Ⅰb, total hysterectomy and double adnexal resection should be done; for stage Ⅰc and above, large omentectomy should be performed at the same time; for advanced stage patients (FIGO stage II and above), tumor cytoreductive surgery should be performed.
2. Tumor cytoreductive surgery Ovarian cancer patients are mostly in advanced stage when they are diagnosed, and at this time, there are extensive metastases in the abdominal cavity, so the primary lesions and metastases should be removed as much as possible, which is called tumor cytoreductive surgery. Generally speaking, retroperitoneal and para-aortic lymph node dissection should be part of the surgery, but many people advocate early lymphatic dissection, for example, in advanced stage with extensive metastasis, retroperitoneal lymph nodes have been completely fixed, so it is difficult to complete this part of the surgery. It is difficult to complete this part of the surgery.
Currently, the standard primary treatment for epithelial ovarian cancer abroad is tumor cytoreductive surgery and postoperative platinum-based combination chemotherapy (the standard chemotherapy regimen is carboplatin + paclitaxel), and more than 80% of patients can achieve clinical remission. Paclitaxel is a new drug discovered only in the 1990s, and platinum-based agents are still mainly used in China because of its high price. The main chemotherapy regimens are PAC regimen (cisplatin + adriamycin + cyclophosphamide), PC regimen (cisplatin + cyclophosphamide), and PT regimen (cisplatin + paclitaxel). For advanced patients who are inoperable, chemotherapy can be given preoperatively to create conditions for surgery.
4. Other therapeutic measures Radiotherapy is rarely applied to epithelial ovarian cancer. The most sensitive ovarian malignancies to radiotherapy are asexual cell tumors. Biological therapy, including immunotherapy and gene therapy, is currently in the research stage and is the direction of future development.
The standard treatment should be tumor cytoreductive surgery and postoperative platinum-based combination chemotherapy. Postoperative chemotherapy is a necessary adjuvant treatment, and the standard chemotherapy regimen is carboplatin + paclitaxel. The patient achieved clinical complete remission, i.e., no evidence of recurrence on gynecological examination, imaging and tumor marker (mainly CA125) monitoring within 6 months after the completion of chemotherapy, and expected to achieve long-term survival.