Неужели диссертацию написали про ТГ вместо пункции?

Думаю, что нет - узнали, что мы почему-то долго и нужно пишем об опредлении ТГ и АТТГ ПОСЛЕ проведения операции и радиоаблации при раке

В файлах в принципе могли поместиться только два слова, поэтому разместили - "рак- ТГ"

Это типичная педагогическая проблема : мы не представляем уровня невежества аудитории, надо было раз 7 сказать : "детки, то , что Вы слышите, относится только к ситуации, когда железа УЖЕ оперирована и пр, а когда она не оперирована, то не надо смотреть ТГ , и вообще надо было стишки написать простенькие ..

Получили базальный кальцитонин: менее 2 пкмоль/мл (надеюсь, не ошиблась в единицах).

Так... пора уходить в управдомы... У меня такое чувство, что маразм начинается и у меня и я так и не застану светлые мгновенья счастья, когда перестану слышать и видеть этот дебилизм.

... И параходы проплувут мимо холмика с одинокой могилкой, протрубив "Салют, Мальчишу"! И паравозы прогудят им во след! И пионеры отдадут салют! ... И найдется какая-то идиотка предпенсионного возраста, которая посмотрит тиреоглобулин или йод в моче и кале или ещё где-то... И повернусь я в этой одинокой могилки вниз подгнившим ликом, дабы не видеть сего чрез пустые мои глазницы!!!
Черт! Черт! Черт!

Черт возьми, что это за люди такие! Откуда они беруться?! Это либо уже клиническая олигофрения, либо это шпионы-вредители.... У меня больше нет объяснений! Неужели есть какой-то третий вариант? Отзовитесь, кто его знает?

Мне позвонили на прошлой неделе из славного города Л ( не буду назвать, чтобы не обидеть другие города)

Им очень ( ну просто очень ) понравилась моя лекция по гиперпролактинемии, поэтому они меня спросить хотят : у них в Л всем смотрят иод в моче, чтобы узнать, нет ли гипотироза, так вот, одна мысль мучает : какие нынче у нас в Москве нормы и какие цифры при гипотирозе?

Терпи, Фадеев..

Аня, на всякий случай, наблюдение за пациентами с медуллярным раком:
[Ссылки доступны только зарегистрированным пользователям ]
pages: 26 - 27.

Review
Medullary thyroid carcinoma
Sophie Leboulleux, Eric Baudin, Jean-Paul Travagli and Martin Schlumberger, Institut Gustave Roussy, Villejuif, France
[Ссылки доступны только зарегистрированным пользователям ]
Should be free.

Яна, спасибо. Многое прояснилось

Review
Medullary thyroid carcinoma
Sophie Leboulleux, Eric Baudin, Jean-Paul Travagli and Martin Schlumberger, Institut Gustave Roussy, Villejuif, France
[Ссылки доступны только зарегистрированным пользователям ]
If not free.


Postoperative management

After total thyroidectomy, thyroxine treatment is given to maintain serum TSH concentration within the normal range.

When measured 5 days after surgery, basal CT concentrations may still be elevated in patients with high preoperative CT concentrations (Fugazzola et al., 1994) and may normalize later. Similarly, CEA has a long half-life in blood. Consequently, serum CT and CEA are measured 2–3 months after surgery: if basal CT is then undetectable, a pentagastrin stimulation test is performed. Patients with no detectable calcitonin concentrations with pentagastrin stimulation testing are likely to be free of disease. However, serum CT again became detectable during follow-up in 5% of these patients (Modigliani et al., 1998; Kebebew et al., 2000). This underlines the need for long-term clinical and biological follow-up.

In patients with clinical disease, biochemical cure is obtained in 75–90% of patients without lymph node involvement, but even with aggressive surgery, in only 20–30% of patients with lymph node metastases, and rarely (4%) in patients with more than 10 lymph node metastases (Scollo et al., 2003). This clearly demonstrates how treatment at an early stage is a highly significant prognostic factor in MTC patients.

In patients with persistently elevated CT levels, the challenge is finding the site of residual disease (Fig. 3). Tumour localization techniques include ultrasonography of the neck and liver, CT or MRI of the neck, chest and liver, and bone scintigraphy. Isotopic scanning with thallium 201, dimercaptosuccinic acid labelled with 99mTc, 123I MIBG, somatostatin analogues labelled with Indium 111 (Baudin et al., 1996) or monoclonal antibodies directed against CT or CEA, and even positron emission tomography (PET) with FDG are poorly sensitive. The use of fluoro-dopamine PET may be more sensitive (Gourgiotis et al., 2003). Liver metastases may be difficult to visualize by these imaging modalities and may be seen only at laparoscopy.

Selective venous sampling catheterization with CT measurements appears to be the most sensitive and specific technique for localizing occult metastatic disease (Wells et al., 1982; Frank-Raue et al., 1992; Abdelmoumene et al., 1994). At the Institut Gustave-Roussy, Villejuif, it is performed in MTC patients with detectable postoperative CT levels below 500 ng/l and no evidence of distant metastases at imaging. Patients with CT levels higher than 500 ng/l are likely to have distant metastases. A gradient in the suprahepatic veins suggests metastatic disease in the liver and may avoid re-operation. In the absence of evidence of distant spread, gradients in the neck or in the mediastinum pinpoint neoplastic foci that need to be removed. Re-operation allowed the removal of neoplastic tissue and a subsequent decrease in CT levels in most patients. It is likely that postoperative CT level will not decrease significantly in patients with thyroid tumour extension beyond the thyroid capsule (pT4) or with more than 10 lymph node metastases or with large mediastinal involvement in the primary operative specimen (Tisell et al., 1986; Abdelmoumene et al., 1994).

This strategy is not unanimously advocated, because the 5-year relapse-free survival was only 60% (Pellegriti et al., 2003) and was similar to that reported with a more conservative approach (Van Heerden et al., 1990). Therefore, in some centres, only patients with palpable or easily visualized lymph node metastases are operated.

External radiation therapy

External radiation therapy (50 Gy in 25 sessions and 5 weeks) to the neck and mediastinum appeared to be effective in two selected groups of patients: in some patients with inoperable tumours it induced long-term stabilization; it reduced, by a factor of 2–4 the risk of neck recurrences in patients with persistently elevated CT after what appeared to be complete surgery (Schlumberger et al., 1991; Brierley et al., 1996; Fersht et al., 2001).

Long term Follow-up

Follow-up protocol

After initial treatment, follow-up is performed every 6 months for 2 years and annually thereafter. It includes serum CT and CEA measurements (Ball et al., 2000; Gagel & Marx, 2002). The probability of finding a recurrence with imaging techniques increases with higher serum CT or CEA concentrations. CT concentration may fluctuate by 20–30% at short-term intervals. As previously discussed, when serum CT and CEA concentrations are elevated, a complete work-up is performed. In the absence of detectable lesions, another work-up is performed after an interval of 6 months to 1 year.

A detectable CT level is compatible with long-term survival, during which CT may remain stable with time or slowly increases. Among patients with detectable CT, a high serum CEA or a rapid increase in CEA concentration signals disease progression.

Screening for pheochromocytoma and hyperparathyroidism is performed in all patients with hereditary MTC, at an interval that is related to the risk.

Local and regional recurrences
Surgery is the main treatment for local and regional recurrences. The extent of surgery depends on the type of previous surgical procedures and on the nature of the recurrence: if initial surgery was complete, recurrent disease is resected; if the extent of initial surgery was incomplete, the primary surgery protocol is completed.

In the absence of known distant metastases, external radiation therapy to the neck and mediastinum may be indicated after surgery when tumour markers remain elevated, which is generally the case.

Distant metastases
Clinical presentation
Distant metastases are the main cause of MTC-related death. Half are present initially. They are often multiple in involved organs and simultaneously affect multiple organs, such as the liver, lungs and bones (Schlumberger et al., 1991).

Lung metastases are macro- or micro-nodular and are generally diffuse throughout both lungs. Bone metastases are osteolytic or osteoblastic on radiographs and bone scintigraphy shows increased uptake. Liver metastases are hyperechoic at ultrasonography and when they are small, they present the same features as hepatic haemangiomas; CT scan, or preferably MRI with injection of contrast medium, may be helpful to confirm the presence of liver metastases (Dromain et al., 2003).

Survival after the discovery of distant metastases is about 20% at 10 years. A few patients with metastatic disease have been long-term survivors, even without any systemic treatment.

Treatment of metastatic disease
Management of metastatic disease is first orientated towards the relief of symptoms. Loperamide is used to treat diarrhoea, and usual treatment modalities are used for pain.

Bone surgery is indicated in patients with orthopaedic or neurological complications or a high risk of such complications. Surgery may also be indicated in case of a single or a few metastases located in the brain, lungs or liver. However, metastases are often diffuse and multiple.

External radiation therapy is indicated for bone metastases not amenable to surgery, especially when they are painful or located in the spine, the base of the skull, and in pelvic or long bones. External radiation therapy procures rapid relief of bone pain and slower recalcification of lesions. It can also be useful for brain metastases. Embolization of bone metastases and injection of cement can efficiently counteract pain, and can be repeated.

In case of liver metastases from other endocrine tumours, embolization or chemoembolization has proven efficient for both symptoms and tumour masses. It may be beneficial in MTC patients with predominant liver involvement, and is more efficient when metastases are smaller than 3 cm and when liver involvement is less than 30% (Roche et al., 2003).

Treatment with radioactive iodine is pointless, because C cells do not take up radioiodine. Radioactive iodine covalently linked to MIBG offers no significant benefit. Treatment with bi-specific antibodies directed against CEA and DTPA and with DTPA labelled with 131I appeared poorly effective in patients with large metastases (Kraeber-Bodéréet al., 2003). Treatment with somatostatin analogues labelled with Yttrium 90 or other radionuclides of patients with high metastatic uptake on octreoscan are still under evaluation.

When high-dose doxorubicin (75 mg/m2 every 3–4 weeks) is used as a single agent or in association with cisplatin, the response rate is probably less than 20%, and major toxic effects were observed (Schlumberger et al., 1991). Furthermore, all responses were partial and short-lived.

MTC is a well-differentiated endocrine tumour, and MTC patients have been treated with drugs demonstrated to be active in other well-differentiated endocrine tumours. No tumour responses have been obtained with etoposide. Various combinations of 5-fluorouracil (5-FU), dacarbazine, streptozocin, cyclophosphamide, vincristine have produced similar response rates (about 20%) with symptomatic improvement in some patients, but no benefit was found on survival rate (Schlumberger et al., 1995). The addition of doxorubicin did not improve the response rate but resulted in higher toxicity (Nocera et al., 2000). The use of chemotherapy should therefore be limited to the few patients with rapidly progressive metastatic disease, who should then be included in prospective controlled trials. In more typical patients with stable or slowly progressive disease, the regimens currently available appear to offer little benefit, if any.

Somatostatin analogues used either alone or in combination interferon α2b simply produced an inconstant and transient effect on diarrhoea. They did not produce any tumour regression and their use is not be recommended in metastatic MTC patients (Modigliani et al., 1992).

The identification of activating mutations of the RET gene has led to efforts to identify inhibitors of tyrosine kinase or of the message transduction pathway, some of which are currently investigated in clinical trials.
[Ссылки доступны только зарегистрированным пользователям ]

Нельзя смотреть только базальный уровень кальцитонина. Необходимо посмотреть еще 1 - 2 точки стимулированного. Именно он имеет прогностическое значение. Пусть не пугает слово "пентагастрин" и вопрос, где его взять. Тысячу раз писал про это, повторюсь еще. Стимуляция осуществляется путем внутривенного введения 20 мл 10% раствора глюконата кальция. Все!!! Т.е. взяли кровь на базальный кальцитонин, потом в/в ввели глюконат кальция и через 5 и 10 минут еще дважды взяли крови на стимулированный кальцитонин.

В догонку....
Помню, на глаза попалась статья 70-ых прошлого века, где выброс кальцитонина стимулировали пероральным приемом виски.
Вот такая наука мне нравится.

Вот почитал Хирургическую литературу,читая описание случая почему -то сразу подумалось о MEN 2 ,здесь вот выдержка из Шварца ,возможно вы это уже хорошо знаете
Medullary Carcinoma

MTCs account for about 5% of thyroid malignancies and arise from the parafollicular or C cells of the thyroid, which, in turn, are derived from the ultimobranchial bodies.43 These cells are concentrated superolaterally in the thyroid lobes, which is where MTC usually develops. C cells secrete calcitonin, a 32-amino-acid polypeptide that functions to lower serum calcium levels. In some animals, especially those that lay eggs with shells, calcitonin is a significant regulator of calcium metabolism, but in humans, it has only minimal physiologic effects.

Most MTCs occur sporadically. However, approximately 25% occur within the spectrum of several inherited syndromes such as familial medullary thyroid cancer, MEN2A, and MEN2B. All these variants are known to result secondary to germline mutations in the RET proto-oncogene. The syndromes are also characterized by genotype–phenotype correlations, with specific mutations leading to particular clinical manifestations. Table 37-8 outlines the salient clinical and genetic features of these syndromes. Figure 37-27 shows some of the clinical features of MEN2B patients.



Features of MEN2B: thickened lips (A) and mucosal neuromas (A and B).

Patients with MTC often present with a neck mass that may be associated with palpable cervical lymphadenopathy (15 to 20%). Local pain or aching is more common in patients with these tumors, and local invasion may produce symptoms of dysphagia, dyspnea, or dysphonia. Distant blood-borne metastases to the liver, bone (frequently osteoblastic), and lung occur later in the disease. The female:male ratio is 1.5:1. Most patients present between 50 and 60 years of age, although patients with familial disease present at a younger age. Medullary thyroid tumors secrete not only calcitonin and carcinoembryonic antigen (CEA), but also other peptides such as calcitonin gene-related peptide (CGRP), histaminadases, prostaglandins E2 and F2, and serotonin. Patients with extensive metastatic disease frequently develop diarrhea, which may result from increased intestinal motility and impaired intestinal water and electrolyte flux. Approximately 2 to 4% of patients develop Cushing's syndrome as a result of ectopic production of adrenocorticotropic hormone (ACTH).

Pathology

MTCs are typically unilateral (80%) in patients with sporadic disease, and multicentric in familial cases, with bilateral tumors occurring in up to 90% of familial patients. Familial cases are also associated with C-cell hyperplasia (Fig. 37-28), which is considered a premalignant lesion. Microscopically, tumors are composed of sheets of infiltrating neoplastic cells separated by collagen and amyloid. Marked heterogeneity is present; cells may be polygonal or spindle-shaped. The presence of amyloid is a diagnostic finding, but immunohistochemistry for calcitonin is more commonly used as a diagnostic tumor marker. These tumors also stain positively for CEA and CGRP.

C-cell hyperplasia, often found in patients with hereditary forms of medullary thyroid cancer.



Diagnosis

The diagnosis of MTC is established by history, physical examination, raised serum calcitonin or CEA levels, and FNA cytology of the thyroid mass. Attention to family history is important because approximately 25% of patients with MTC have familial disease. Because it is not possible to distinguish sporadic from familial disease at initial presentation, all new patients with MTC should be screened for RET point mutations, pheochromocytoma (24-hour urinary levels of VMA, catecholamine, and metanephrine), and hyperparathyroidism (serum calcium). It is important to rule out a coexisting pheochromocytoma to avoid precipitating a hypertensive crisis and death. Screening of patients with familial MTC for RET point mutations has largely replaced using provocation testing with pentagastrin or calcium-stimulated calcitonin levels to make the diagnosis. Calcitonin and CEA are used to identify patients with persistent or recurrent MTC. Calcitonin is a more sensitive tumor marker, but CEA is a better predictor of prognosis.

Treatment

If patients are found to have a pheochromocytoma, this must be operated on first. These tumors are generally (>50%) bilateral. Total thyroidectomy is the treatment of choice for patients with MTC because of the high incidence of multicentricity, the more aggressive course, and 131I therapy is not usually effective. The central compartment nodes are frequently involved early in the disease process, so that a bilateral central neck node dissection should be routinely performed. In patients with palpable cervical nodes or involved central neck nodes, ipsilateral or bilateral, modified radical neck dissection is recommended. Similarly, patients with tumors larger than 1.5 cm should undergo ipsilateral prophylactic modified radical neck dissection, because greater than 60% of these patients have nodal metastases. Approximately 30% of these patients will also have contralateral nodal metastases. In the case of locally recurrent or metastatic disease, tumor debulking is advised, not only to ameliorate symptoms of flushing and diarrhea, but also to decrease risk of death from recurrent central neck or mediastinal disease. External beam radiotherapy is controversial, but is recommended for patients with unresectable residual or recurrent tumor. There is no effective chemotherapy regimen. Radiofrequency ablation done laparoscopically appears promising in the palliative treatment of liver metastases larger than 1.5 cm. Tumors that express c-kit may also respond to tyrosine kinase inhibitors such as Gleevec.

In patients who have hypercalcemia at the time of thyroidectomy, only obviously enlarged parathyroid glands should be removed. The other parathyroid glands should be preserved and marked in patients with normocalcemia as only approximately 20% of patients with MEN2A develop hyperparathyroidism. When a normal parathyroid cannot be maintained on a vascular pedicle, it should be removed, biopsied to confirm that it is a parathyroid, and then autotransplanted to the forearm of the nondominant arm in MEN2A patients and in the sternocleidomastoid muscle in other patients.

Total thyroidectomy is indicated in RET mutation carriers once the mutation is confirmed. The procedure should be performed before age 6 years in MEN2A patients and prior to age 1 year in MEN2B patients.44 Central neck dissection can be avoided in children who are RET positive and calcitonin negative with a normal ultrasound examination. When the calcitonin is increased or the ultrasound suggests a thyroid cancer, a prophylactic central neck dissection is indicated.

Postoperative Follow-Up and Prognosis

Prognosis is related to disease stage. The 10-year survival rate is approximately 80% but decreases to 45% in patients with lymph node involvement. Survival also is significantly influenced by disease type. It is best in patients with non-MEN familial MTC, followed by patients with MEN2A, and then by patients with sporadic disease. Prognosis is the worst (35% at 10 years) in patients with MEN2B. Patients with tumors that stain poorly for calcitonin and with a heterogeneous distribution of calcitonin do worse than patients in whom calcitonin staining is increased and homogeneous. Performing prophylactic surgery in RET oncogene mutation carriers not only improves survival rates, but also renders most patients calcitonin free.

Aлександр, а что в описании случая вас заставило думать о MEN2?
Кстати, Аня, ей, наверное, можно дозу тироксина уменьшить. Ей ведь не нужна супрессивная терапия.

В данном конкретном описании случая - действительно нет никакой специфической информации позволяющей об этом состоянии подумать. .Однако в моей голове сразу как-то автоматически сразу всплыл MEN 2,как только Анна упомянули диагноз медуллярной карциномы.
Согласитесь ,было бы интересно детализировать семейный анамнез , есть ли история гипертензии ?Уж не знаю насколько практично в России определение RET .Как-то элегантнее ,что ли,выглядел бы разбор интересного случая. История просто напомнила подобный case произошедший лет 6 назад. В любом случае коллеги не судите строго ,я не эндокринолог ,высказал собственное суждение.

Согласен с Вами. При выявленном МРЩЖ необходимо исключать наследственный характер патологии, равно как и возможные комбинации - МЭН 2А и 2Б типа (причем, это необходимо делать при отсутствии любых клинических проявлений фео). Семейный анамнез, безусловно, очень важен, но нужно помнить, что данный клинический случай может быть представлен первой/новой мутацией RET. Поэтому в диагностический алгоритм обязательно надо включить исследование мутаций RET и определение метилированных производных катехоламинов в крови/моче. Помимо всего прочего характер мутации RET играет важную роль в прогнозировании результатов лечения МРЩЖ.

ЗЫ: Практически каждый случай МЭН 2Б легко заподозрить по внешнему виду больного с МРЩЖ.