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A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia.
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Hormonal study and dynamic biochemical tests performed indicated ECS.
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Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases.
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Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis.
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The patient underwent right parotidectomy, and histopathologic examination confirmed ACC.
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Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide.
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Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed.
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We present a case of a 32-year-old woman with a history of gradual enlargement of the anterior neck.
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The medical history was unremarkable and no comorbidities existed.
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There was no history of radiation exposure.
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Clinical examination revealed a multinodular goiter.
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Computed tomography showed a thyroid nodule of the left lobe extended to the isthmus and the right lobe with anterior and posterior capsular rupture contracting close contact with the vascular axis left carotid-jugular plunging into the cervicothoracic away from the hole aortic arch.
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There was no evidence of lung lesions.
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The patient underwent a surgical exploration.
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There was a hard left lobe nodule of 5 cm infiltrating the adjacent muscles and partially infiltrates the trachea.
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The intraoperative consultation pathology diagnosis was: undifferentiated carcinoma.
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A total thyroidectomy was realized.
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Histological examination showed a proliferation of elongated spindle-shaped cells, arranged in interweaving fascicles of varying sizes, intersected at right angles.
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Tumor cells are atypical with strange nuclei.
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Chromatin is distributed inhomogeneously.
[ "Chromatin", "is", "distributed", "inhomogeneously", "." ]
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The cytoplasmic membrane is irregular and thick.
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The nucleolus is very large.
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The tumor realize a pushing against thyroid parenchyma which is separated with a fibrous capsule.
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The mitotic rate was extremely high (19 mitosis/10 high power field), and atypical mitotic figures were also present.
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The neoplasia showed invasion of the peri-glandular fat tissue.
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Immuno-histochemical staining of the slides with caldesmon, desmin, PanCK, CK5-6, CK7, myogenin, epithelial membrane antigen (EMA), CEA, thyroid transcription factor (TTF-1), pancytokeratin, smooth muscle actin (SMA), MelanA, S 100 protein, CD 45, CD3, CD30, CD 20, CD 15, CD34, ALK, calcitonin and KI 67 protein was performed.
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The tumour was strongly positive for caldesmon, SMA, desmin, and negative for pancytokeratin and other epithelial, lymphoid and melanocytic markers.
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On the basis of the clinical, radiographic, histopathological and immunohistochemical features, the final diagnosis was primary thyroid leiomyosarcoma, FNCLCC grade 3.
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In multidisciplinary tumour board, it was decided that adjuvant loco regional RT and chemotherapy by ifosfamide and doxorubicin.
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A 17 months girl have been consulted in our service for colicky abdominal pain often associated with constipation and loss of appetite, since she was six months old.
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Parents kept her at home until they remarked visible peristalsis on the abdominal wall, which lead them to consult our service.
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She was born from a full-term and uncomplicated pregnancy during which the mother had no prenatal ultrasound (US).
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Her neonatal period was unremarkable.
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Clinical findings: on physical examination, she was colored and active.
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Her vitals were within normal limits as well as her anthropometric parameters (weight: 10kg).
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Her abdomen had moderate distension and peristaltic waves were visible on the abdominal wall.
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Abdomen was soft on palpation and tympanic on percussion.
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The digital rectal examination was unremarkable.
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Symptomatic treatment was initiated with lactulose (five mL bid) and an antispasmodic, trimebutine (five mL bid).
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Timeline: our patient experienced first symptoms since August 2019.
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Parents brought her at our service only on 27 th July 2020 two days after they remarked visible peristalsis on her abdominal wall.
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The patient has been treated and discharged in August 2020 and pathology results were available 11 days later.
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Diagnostic assessment: blood results were within normal range.
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Abdominal X-ray showed distension of an intestinal loop in the left lumbar region.
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Abdominal US identified a misleading colonic dilatation containing heterogeny echoic substance, with no other additional features.
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Contrast enema was unremarkable.
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Due to financial constraints, upper gastro-intestinal (GI) series and abdominal computed tomography (CT) could not be performed.
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An exploratory laparotomy has been indicated with presumptive diagnosis of intestinal duplication.
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Therapeutic intervention: after the median laparotomy, a dilatated segment of the jejunum was identified at approximately ten cm from the Treitz's angle.
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Its diameter was fourfold greater than of the adjacent bowel, to which the dilatation was connected abruptly, without any identified obstructive cause.
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It measured ten cm in length and had prominent serosal vessels.
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Adjacent mesentery was thickened.
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No further anatomic anomaly was identified.
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A resection of the dilatated portion was performed, followed by end-to-end jejunal anastomosis using Vicryl 3/0 in separate stitches.
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Follow-up and outcomes: microscopic examination revealed a lymphocytic infiltration of both layers of the muscularis propria.
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The dense lymphocytic infiltration was mainly constituted of T lymphocytes CD3+/8+ (C and D) and some B lymphocytes CD20+.
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Locally diminished actin coloration indicated atrophy of smooth muscle fibers.
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Nerve fibers and ganglion cells of myenteric and submucosal plexuses were intact with focalized absence of NK CD56+ cells around lymphocytic infiltration.
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No ectopic tissue was identified.
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The postoperative period was uneventful.
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The patient was on parenteral nutrition until day seven postoperatively.
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She was discharged on day ten and after three months of monthly follow-up, colicky abdominal pain and chronic constipation had disappeared since the early postoperative days.
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For the future, the follow-up will be done on a three months basis for a year and then, twice yearly.
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Here we report a case of aggressive metastatic cholangiocarcinoma (MCC) in 72-year-old man, sequentially treated with two targeted chemotherapies.
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Initially disease quickly progressed during best clinical practice care (gemcitabine in combination with cisplatin or capecitabine), which was accompanied by significant decrease of life quality.
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Monotherapy with TKI sorafenib was prescribed to the patient, which resulted in stabilization of tumor growth and elimination of pain.
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The choice of the inhibitor was made based on high-throughput screening of gene expression in the patient's tumor biopsy, utilized by Oncobox platform to build a personalized rating of potentially effective target therapies.
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However, time to progression after start of sorafenib administration did not exceed 6 months and the regimen was changed to monotherapy with Pazopanib, another TKI predicted to be effective for this patient according to the same molecular test.
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It resulted in disease progression according to RECIST with simultaneous elimination of sorafenib side effects such as rash and hand-foot syndrome.
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After 2 years from the diagnosis of MCC the patient was alive and physically active, which is substantially longer than median survival for standard therapy.
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A 50-years-old woman, hypertensive, hospitalized for a large cervical mass appeared 30 years ago.
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In the family history, her mother, sisters and cousins underwent a surgery for MNG.
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Despite of the large volume of the mass, the patient never described signs of cervical compression whatsoever respiratory, digestive, laryngeal, vascular or neurologic signs.
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She never suffered from thyroid dysfunction.
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Her neck was deformed by the voluminous formation classified grade III according to the WHO modified classification.
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The mass took the front and the two sides of the neck.
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Its surface was embossed and covered by a thin normal skin.
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There were some veins of the collateral circulation limited to the neck.
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The goiter measured 18 x 11 cm.
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The mass was firm, painless, and mobile with the swallowing movements.
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Lymphadenopathy research was difficult and found no palpable lymph nodes.
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The laboratory tests (T 3, T 4 and TSH) were normal.
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Thoracic radiography showed a large cervical opacity roughly round and strewn with microcalcifications associated with a right eccentricity of the trachea.
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Cervical and chest CT revealed the presence of a partially calcified thyroid mass slightly plunging in the anterior mediastinum.
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It took heterogeneously the contrast and then evocate a large MNG.
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The trachea was surrounded by the goiter, slightly narrowed and right deviated as well as the lower part of the larynx.
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The right and left vascular axes of the neck (carotid artery and jugular vein) were deviated backward.
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The patient underwent a surgery for her enormous MNG slightly plunging in the mediastinum.
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Endotracheal intubation was relatively easy by the laryngoscope.
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The incision performed was a Kocher cervicotomy.
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There was a multinodular, hypervascularized goiter.
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Its lower end plunges behind the sternal manubrium.
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The larynx was deviated towards the right side.
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The total thyroidectomy was performed in two steps: initially a right lobo-isthmectomy, then the left lobectomy.
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The retrosternal part of the goiter was released using the finger by the same incision.
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Both recurrent laryngeal nerves (RLN) were not identified because of the hemorrhage.
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One parathyroid gland was accidently devascularized and was autotransplanted to the ipsilateral sternocleidomastoid muscle.
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The operation was finished by double aspiration drainage.
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In the first hours after surgery, the patient developed a large cervical hematoma.
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She was readmitted to the operating room, and after evacuation of the hematoma there was no vessels bleeding.
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