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In the absence of clear interstitial invasion, encapsulated papillary carcinoma (EPC) of the breast may be attributed to an extremely good prognosis if handled similarly to ductal carcinoma in situ (DCIS) with suitable local treatment. Here, we report our experience with a case of EPC of the breast that presented with carcinomatous pleuritis and lymphangitis carcinomatosa postoperatively, which rapidly resulted in a poor outcome. A 67-year-old woman was diagnosed with DCIS of the left breast and underwent left partial mastectomy and sentinel lymph node biopsy. EPC was diagnosed because the pathological examination showed no sign of interstitial infiltration. Postoperative radiation therapy was performed. Five years and 9 months postoperatively, the patient began experiencing cough and shortness of breath on exertion. Imaging showed right pleural effusion and consolidation of the lung field, but nothing suggesting local recurrence in the preserved left breast, local lymph nodes, or opposite breast was observed. Postoperative recurrence of breast cancer, carcinomatous pleuritis, and lymphangitis carcinomatosa were diagnosed based on the results of pleural fluid cytology. One month later, multiple brain metastases were found, and the patient died of the primary disease 5 months after recurrence. After surgery for EPC without clear interstitial infiltration, there was a small possibility of a poor outcome from distant metastasis. Therefore, although distant metastasis is uncommon, regular examination and testing should be performed.

Background: Dyspnea is a common and distressing symptom in patients with advanced cancer. Opioids, benzodiazepines, and corticosteroids are commonly prescribed pharmacological treatments for cancer dyspnea. Objective: The objective of this survey was to investigate physician-perceived predictive factors for the effectiveness of opioids, benzodiazepines, and corticosteroids in treating cancer dyspnea. Design: This study involves a nationwide survey using self-report questionnaires. Setting/Subjects: Random sampling selected 268 Japanese certified palliative care physicians in Japan. Measurements: We inquired about the 12 physician-perceived predictive factors for the effectiveness of drugs (opioids, benzodiazepines, and corticosteroids) in treating cancer dyspnea. Results: The frequently selected physician-perceived predictive factors for the effectiveness of opioids were tachypnea, respiratory effort, opioid naive, Eastern Cooperative Oncology Group Performance Status 0-2, multiple lung tumors, dry cough, pleural effusion, and pleural lesion. Benzodiazepines were predicted to be effective against dyspnea in patients with depression and severe anxiety. Meanwhile, corticosteroids were predicted to be effective against dyspnea in patients with lymphangitis carcinomatosa, superior vena cava syndrome, major airway obstruction, and audible wheezing. Japanese palliative care physicians anticipate that different drug classes will be effective for treating dyspnea in patients with specific factors. Conclusions: Japanese palliative care physicians expect that different drugs will be effective for dyspnea in patients with specific predictive factors. Future prospective studies are required to assess the effectiveness of each drug class against specific dyspnea.

Pulmonary lymphangitis carcinomatosis (PLC) is a life-threating complication in patients suffering from malignancies. Misleading and nonspecific symptoms often result in a delayed diagnosis. This review was aimed at evaluating epidemiology, clinical manifestations, and survival of patients with PLC reported in the literature. According to our knowledge, this study is the first such extensive analysis of PLC. We searched for the literature in the PubMed database for articles published from 1970 to 2018 using keywords: lung, pulmonary, lymphangitic, carcinoma, carcinomatosis. Pulmonary lymphangitis carcinomatosis rarely occurs, thus all data were extracted from case reports and case series consisted of a method for identifying individual-level patient data. In the final analysis, 108 articles (139 individual patient cases) were included. The mean age of PLC occurrence is 49.21 years. There is no difference in the prevalence between men and women. The most common underlying primary tumors coexisting with PLC are breast (17.3%), lung (10.8%), and gastric cancers (10.8%). Dyspnea and dry cough were the most common symptoms occurring in 59.0% and 33.8% of patients, respectively. In half the patients, PLC developed in fewer than ten months after first diagnosis of cancer. Pulmonary lymphangitis carcinomatosis diagnosis is associated with a poor prognosis: approximately half of patients die within two months of their first respiratory symptoms and three weeks from admission to hospital. Regarding survival time, we observed better results achieved in patients described between 2000 and 2018 compared to 1970 through 1999. In the presence of progressive dyspnea, cough, and lesions comparable to interstitial lung disease, diagnosis of PLC should be considered. Pulmonary lymphangitis carcinomatosis can be the first manifestation of primary occult neoplasm and may occur at any age. Adenocarcinoma, especially primary lung, breast, and gastric cancers is the most common cancer coexisting with PLC.

The hypoxic patient with a normal chest X-ray can be a diagnostic challenge. This case illustrates the rational diagnostic process and describes a relatively rare but important complication of cancer metastasis. Thrombotic microangiopathy, like lymphangitis carcinomatosa, may cause respiratory failure and is a poor prognostic finding. However, unlike lymphangitis carcinomatosa, it may not have specific findings on cross-sectional imaging.

After the liver, the lung is the second most common site for metastatic disease (Treasure et al., Thorax 69:946–9, 2014). Consequently, 25–55% of patients with malignancy will develop lung metastases at some point during the course of their disease (Crow et al., Cancer 47:2595–602, 1981; Johnson and Lindskog, JAMA 202:94–8, 1967; Abrams et al., Cancer 3:74–85, 1950). The rich capillary network of the lung and its central location in the circulatory system predispose the lung to the arrest and implantation of circulating tumor cells in the terminal pulmonary vasculature with subsequent spread via direct extension or lymphatic spread into adjacent structures. The diagnosis of pulmonary metastatic disease is usually not a major challenge, especially when supported by an appropriate clinical history with known malignancy elsewhere and radiologic evidence of multiple lung nodules. However, distinction of a solitary pulmonary metastasis from a primary bronchogenic carcinoma, especially after a prolonged disease-free interval may be more difficult and usually requires a multidisciplinary approach, including tissue biopsy, for definitive diagnosis. In this chapter, the pathogenesis, patterns of spread, most common metastatic tumor types, and mechanisms of pulmonary metastasis will be discussed.

Highlights • Lymphangitis carcinomatosa is known as commitment of pulmonary lymph vessels. • Intestinal lymphangitis carcinomatosa is rarely reported. • This is the first report of intestinal lymphangitis related to ovary cancer. • This is the first report of intestinal lymphangitis other than duodenal., We present a 57-year-old woman with ovarian cancer that presented to the Emergency Room with a proximal small bowel obstruction. Exploratory laparotomy evidenced a thickened 10 cm extension of the proximal jejunum without bowel peristalsis, with stenotic enteric lumen, with a lesion apparently originating from its submucosal and muscular layers. The patient underwent an exploratory laparotomy with total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, small bowel resection and peritoneal biopsies. Final pathology and immunohistochemistry confirmed the intra-operative suspicion of lymphatic intestinal spread of malignant cells originating from a high grade serous carcinoma of ovarian origin. To the best of our knowledge, this is the first report in the literature of intestinal carcinomatous lymphangitis related to ovarian cancer, and the first report of involvement of the proximal portion of the jejunum.

Abstact Rationale: Metastatic gastric cancer patients with poor eastern cooperative oncology group performance status (PS) (≥3) were lack of effective anti-tumor strategies. They always lived with poor PS, severe and multiple symptoms, and usually resulted in extremely limited survival time. Herein, we reported a patient diagnosed with gastric cancer metastasized to multiple bones, along with lymphangitis carcinomatosa in lungs, harboring Her-2 and c-MET amplification with poor PS, positively responded to combinational therapy with trastuzumab and crizotinib. Patient concerns: The patient complained of persistent cough and fatigue for 2 months, otherwise, she denied smoking, alcohol history, or any other medical or family history. Diagnosis: With the biopsy results from gastroscopy, as well as computer tomography for chest and abdomen, the patient was diagnosed as gastric adenocarcinoma, with metastasis on lungs, left adrenal gland, retroperitoneal lymph nodes, and multiple bones. Interventions: Because of the poor PS (PS = 3), as well as Her-2 and c-MET amplification, the patient received combination treatment with trastuzumab and crizotinib as salvage strategy. Outcomes: After 2 months’ exposure of trastuzumab and crizotinib, symptoms including persistent cough, and chest distress were alleviated significantly. Simultaneously, chest computer tomography showed significant dissipation of lymphangitis carcinomatosa, as well as apparent reduction of pleural effusion. No adverse reactions including nausea, vomiting, diarrhea, or hypertension was observed during the following 2 months. Lessons: The present case suggested that combinational therapy with trastuzumab and crizotinib might be effective in metastatic gastric cancer patients harboring Her-2 and c-MET amplification, even with a poor PS. It was also implied that gene sequencing might be valuable, especially in patients with limited treatment strategies.

Acute kidney injury (AKI) is a major complication in patients with cancer, associated with significant morbidity and mortality. Only two cases of kidney lymphangitic carcinomatosis associated with AKI have been reported, in gastric and colorectal adenocarcinoma. Here, we report on a 53-year-old man with pancreatic adenocarcinoma who developed AKI as a result of kidney lymphangitic carcinomatosis. The patient rapidly became anuric and required haemodialysis. Kidney lymphangitic carcinomatosis should be considered as a cause of AKI in patients with cancer and may become a more frequent clinical finding as patients with metastatic carcinoma survive for longer.

Background Globally, cervical cancer is the fourth most common cancer in women. Here, we report a case of cutaneous lymphangitis carcinomatosa arising from cervical cancer, an extremely rare and treatment-resistant condition. Case presentation A 64-year-old Japanese woman presented with genital bleeding. She was diagnosed as having stage IB1 squamous cell cervical cancer and subsequently treated with radiotherapy. Approximately 2 years after the curative radiotherapy, she developed itching, skin rash, and small nodules on her left femoral and pubic area. Slight 18F-fluorodeoxyglucose uptake was detected at her left femoral skin on positron emission tomography with computed tomography. A histopathological examination was performed on a biopsy sample from an erythematous macule on her left femoral skin and vulva. Consequently, she was diagnosed as having cutaneous lymphangitis carcinomatosa arising from cervical cancer. Paclitaxel (135 mg/m2), cisplatin (50 mg/m2), and bevacizumab (15 mg/kg) combination therapy was administered every 21 days. Both itching and rash improved after three treatment cycles. After the completion of six cycles, skin erythema in the femoral and vulval area disappeared completely. Our patient experienced a 25-month symptom-free interval after the last chemotherapy session. Conclusion Our findings suggest that combination chemotherapy plus bevacizumab is an effective therapeutic option in patients with cutaneous lymphangitis carcinomatosa arising from cervical cancer.

Objectives To report an intestinal lymphangitis carcinomatosa related to ovary cancer. Methods The information has been obtained through review of medical records and review of the literature. Results A 57-year-old virgin, deaf-mute patient with cognitive deficit was referred to our hospital with left ovary neoplasia. Abdominal CT showed a 3.5 cm diameter mixed lesion in the left ovary. CA-125 was 74.55. Videolaparoscopy showed lymphatic fluid in the abdominal cavity, lesions in epiploon compatible with metastatic implants. Left oophorectomy was performed. Frozen section revealed undifferentiated malignant neoplasia, not being able to rule out lymphoma. Pathology confirmed the hypothesis of high grade serous ovarian carcinoma. She was readmitted in 2 weeks later with clinical signs of high bowel obstruction. Abdominal CT demonstrated proximal jejunal obstruction. Exploratory laparotomy showed a thickened area of 10 cm in the proximal jejunum, with stenotic enteric lumen, with lesion apparently originating from its submucosal and muscular layers without implants in serosa. Proximal enterectomy, panhysterectomy, omentectomy, and resection of peritoneal implants in hepatic round ligament and bladder were performed. Pathology revealed jejunal stenosis due to an infiltrating tumor, lengthening 7.2 cm. Microscopy showed lymphatic tumoral emboli in mucosa, submucosa and muscularis propria. No serosal involvement was identified. Immunohistochemistry was compatible with metastatic high grade serous ovarian carcinoma. Conclusions Our report demonstrates an unusual pattern of metastasis: embolization of intestinal lymphatic vessels, with subsequent stromal invasion. To the best of our knowledge, this is the first report in the literature of intestinal carcinomatous lymphangitis related to ovary cancer.

The diagnostic yield of conventional transbronchial lung biopsy varies among various parenchymal lung diseases: in pulmonary sarcoidosis and lymphangitis carcinomatosa, a diagnosis can be obtained in up to 80% of patients; this method is considered inadequate, however, in identifying more complex histological patterns such as usual interstitial pneumonitis or nonspecific interstitial pneumonitis, mainly because the specimens are tiny and the interpretation is confounded by crush artifacts. Recently, the use of cryoprobes has achieved a significant impact on this issue. This review is about this promising application of cryobiopsy in the diagnostic process of diffuse parenchymal lung diseases.Recent studies document that with transbronchial cryobiopsies, the diagnosis of usual interstitial pneumonitis can be made confidently by pathologists with a good interobserver agreement. Pneumothorax is the main complication (reported in up to one-quarter of cases in some series); bronchial bleeding is usually controlled using Fogarty balloon.Transbronchial cryobiopsy is a promising new technique that may become a valid alternative to surgical lung biopsy in the near feature.

Pulmonary lymphangitis carcinomatosa occurs due to dissemination of cancer cells in pulmonary lymphatics. It is only rarely seen with urological malignancies and portends a grave prognosis. Its presence in patients with urothelial carcinoma of the urinary bladder is uncommon. We present a case of a nested variant of transitional cell carcinoma of the urinary bladder associated with pulmonary lymphangitis carcinomatosa. We also discuss the symptoms and radiological features that might aid in timely diagnosis of this entity.

BACKGROUND: One of the features characterising cutaneous SCC as high-risk is lymphovascular infiltration. The diffuse lymphangitic spread of carcinogenic cells is defined as the so-called lymphangitis carcinomatosa. In some cases, it is the only and first sign to alert the presence of an underlying malignancy. Therefore, biopsy in patients with clinical data on lymphangiosis carcinomatosa is of paramount importance. CASE REPORT: We present a 77-year-old man with a progressively growing tumour formation in the area of the right shoulder, clinically suspected for SCC. During the dermatological examination, it was found that the lesion was surrounded by an infiltrated, perilesional relief shaft, which was histologically verified as lymphangitis carcinomatosa. The tumour formation was removed by radical excision and formation of a large skin-subcutaneous defect. To correct the surgical defect, a cervico-pectoral flap was performed, followed by tunnel transposition of the scapular graft through the deltoid muscle. The preoperative, ultrasound-marked artery was the arteria circumflexa scapulae dextra, which was used as the foot of the scapular graft and at the same time ensuring its blood supply. After the performed surgical flaps there remains a small uncovered surgical defect, which was left for subsequent secondary healing or full thickness mesh graft. The subsequent histological examination of the removed tumour formation detected the presence of squamous cell carcinoma. CONCLUSION: Patients with the simultaneous presence of two different pathological cutaneous changes, located in the immediate proximity often require a multidisciplinary and complex treatment approach. For tumour formations close to the area of the neck, the cervical-pectoral flap provides optimal cosmetic recovery of the surgical defect. The tunnel transposition is an individualised, unconventional and difficult to implement the approach, which however showed a good therapeutic result. On the other hand, the preoperative histological examination of reddish peritumoral localised tentacles leads to 1) diagnosis of lymphangiosis carcinomatosa as well as 2) the subsequent precise determination of the limits of surgical excision, which is a large number of cases saves the need for secondary re-excision in these patients.

In 1963, the first bronchoscopic lung biopsy was performed. Less than 10 years later, the technique of transbronchial lung biopsy using a flexible bronchoscope was introduced into clinical practice, significantly reducing the rate of major complications and the rate of surgical lung biopsies in patients with diffuse parenchymal lung diseases. The diagnostic yield of transbronchial lung biopsy varies among various parenchymal lung diseases. In pulmonary sarcoidosis and lymphangitis carcinomatosa, a diagnosis can be obtained in up to 80% of patients. This method is considered inadequate, however, in identifying more complex histological patterns such as usual interstitial pneumonitis or nonspecific interstitial pneumonitis. Introduction of the 'jumbo forceps' and of a more 'surgically oriented' procedural setting (patients deeply sedated and intubated) allowed larger and more numerous lung specimens to be obtained without a significant increase of complications such as pneumothorax or bronchial bleeding. However, the possibility to obtain enough parenchymal tissue for a morphological diagnosis of complex patterns remained unmet. Recently, the use of cryoprobes has achieved a significant impact on this issue allowing to obtain large quantity of tissue. Recent studies document that with transbronchial cryobiopsies the diagnosis of usual interstitial pneumonitis can be made confidently by pathologists with a good inter-observer agreement. Pneumothorax is the main complication (reported in up to one fourth of cases in some series); bronchial bleeding is easily controlled using Fogarty balloon. Transbronchial cryobiopsy is a promising new technique that may become a valid alternative to surgical lung biopsy in the near feature.

Rationale: Pembrolizumab, an immune-checkpoint inhibitor (ICI), has been shown to be effective for treatment-naive patients with non–small cell lung cancer (NSCLC) and high expression of programmed death-ligand 1 (PD-L1). Therefore, treatment regimens containing pembrolizumab have become a standard therapy for these patients. However, the use of pembrolizumab is limited owing to the side effects of ICIs. Patient concerns and diagnoses: The patient was a 65-year-old man with a left lung mass surrounded by interstitial shadow. The tumor was diagnosed as adenocarcinoma, cT4N3M0, stage IIIC, and the tumor cells showed high PD-L1 expression. It was unclear whether the interstitial shadow was interstitial lung disease (ILD) or lymphangitis carcinomatosa. Interventions and outcomes: The patient received carboplatin and nab-paclitaxel, a less risky regimen for ILD, as the first-line therapy. Administration of 2 cycles of this regimen markedly improved both the tumor diameter and interstitial shadow. The interstitial shadow was clinically diagnosed as lymphangitis carcinomatosa and not ILD. Subsequently, the patient was treated with pembrolizumab, and the tumor showed much further shrinkage with no deterioration of the interstitial shadow. To date, the patient is alive with no complaints and no disease progression, and has continued pembrolizumab treatment for a total of 12 months. Lessons: In patients at a high risk of ICI-related side effects, platinum-doublet chemotherapy may be permitted as the first-line therapy for NSCLC with high PD-L1 expression. However, if the risk associated with ICIs is resolved, early switching from chemotherapy to pembrolizumab might be desirable, even if the chemotherapy is effective.

Dear Editor, Cutaneous metastases (CM) are detected in about 0.6-10.4% of patients with an internal malignancy (1-3). Excluding melanoma, breast and lung carcinomas are the main source of CM in women and men, respectively (1,4,5). CM can have different clinical features, and a diagnosis of CM is usually suspected before performing a biopsy. However, this can be a pitfall for clinicians when the clinical presentation is not the typical inflammatory nodule or mass. Herein we report 2 cases of cutaneous metastases of breast carcinoma, initially treated as a common skin infection. Case 1 A 51-year-old Caucasian woman presented to our Institute with a four-month history of diffuse and erythematous pustular, lesions on the right arm that were painless and non pruritic (Figure 1). The patient had undergone excision for a breast adenocarcinoma (stage IIIA) 5 years earlier. An initial diagnosis of folliculitis was established, and the patient started systemic and topical antibiotics without any improvement. Based on the clinical features and the patient medical history, we performed a skin biopsy. Pathologically dermal nests of tumor cells, arranged in a glandular-like pattern and involving the perifollicular and follicular areas (Figure 2, Figure 3), were highlighted. The tumor cells were positive to cytokeratin (CK) 7, CK19, and carcinoembryonic antigen (CEA) and negative for CK20, CK5/6, CD10, and thyroid transcription factor-1 (TTF-1) (Figure 4). According to the clinical history and pathology, a final diagnosis of folliculotropic metastatic breast carcinoma was established. Unfortunately, the patient died after 10 months. Case 2 A 61-year old Caucasian woman presented to our Department with a two-month history of pink/violet macular lesions with diffuse telangiectasia on the left breast and arm (Figure 5, Figure 6). Five years earlier she had undergone excision for a breast adenocarcinoma (stage II A). A previous diagnosis of cellulitis had been made, and systemic antibiotic therapy had been started without any improvement. Based on the clinical features and the patient medical history, a punch biopsy was performed. Examination of skin biopsy showed a diffuse, sclerotic, and mixoid stroma with several dense ectatic lymphatic vessels (Figure 7, Figure 8). The dermal and hypodermal lymphatic lumens were filled with neoplastic cells. Thus, a diagnosis of cutaneous lymphangitis carcinomatosa (CLC) was established. Unfortunately, the patient died after 8 months. Discussion CM are present after breast carcinoma in about 23.9% of patients, often involving the chest and abdomen and manifesting on average 5 years after surgical removal of the first malignancy (1,6). CM of breast cancer are usually solitary or multiple nodular pinkish lesions (ranging between 1 and 3 cm) (1). However, several clinical features have been reported in the literature, including telangiectatic carcinoma, erythema-like, erythema annulare centrifugum-like, morphea-like, erysipelas-like, dermatofibroma-like, herpes-zoster-like, and alopecia-like lesions (1,7-10). Clinical and pathological images of folliculitis-like metastases are rarely reported in the literature, especially after breast cancer (11,13) Clinically, folliculitis-like metastases could resemble a zosteriform-like metastatic lesion (7,14,15) although they do not follow a dermatome and are pustular lesions rather than violaceous indurate papules and/or nodules (13,14) Pathologically, our cases showed an infiltration of the dermis and pilosebaceous units growing through the pilosebaceous unit in a "pseudo-eruptive way". In this regard, folliculitis-like CM could be similar to alopecia neoplastica, where the metastatic process involves and destroys the pilosebaceous units completely, leading to scarring alopecia (9,10). However, in our case, the pilosebaceous unit was still slightly recognizable, and clinically there were no scar-like features. The mechanism of folliculitis-like metastasis formation is currently unknown. As reported in zosteriform-like metastases, the lymphatic and hematogenous spread of malignant cells or the koebnerization at the site of a previous viral and/or bacterial infection could lead to metastasis (7,14-16). However, unlike zosteriform-like metastases, the spread of neoplastic cells from the dorsal root ganglia was not a plausible mechanism of metastasization in our cases because of the absence of dermatome involvement. Furthermore, there were no signs of possible koebnerization in a previous bacterial and/or viral infection site (7,13) In our opinion, folliculitis-like metastasis may be a result of the skin extruding malignant cells through the pilosebaceous unit to limit the neopalstic proliferation. This could explain the clinical and pathological features of folliculitis-like metastasis. Alternatively, the adnexotropic behavior of malignant cells may be explained by homing mechanisms, involving the up-regulation of the intercellular adhesion molecule 1 (ICAM-1) on the follicular epithelium, such as folliculotropic mycosis fungoides (17). In our patient, the folliculitis-like eruption was the first sign of recurrence after 5 years of disease-free survival. It is evident that the unusual folliculitis-like eruption of CM led to a delay in the diagnosis. CLC is a rare presentation of skin metastasis, characterized by an occlusion of dermic lymphatic vessels by neoplastic cells (18). CLC has been reported in the literature in association with several malignancies, including lung, breast, and ovarian cancer (19). CLC shows pink/violet macular lesions with diffuse telangiectasias, often associated with itching and burning sensation. The main differential diagnoses are erysipelas and cellulitis. However, CLC is not associated with fever, chills, and leukocytosis. Furthermore, CLC shows no response to antibiotic therapies. Several clinicopathological types of cutaneous metastasis have been reported in the literature, including telangiectatic metastatic breast carcinoma (TMBC) and carcinoma erysipelatous (CE). TMBC is characterized by yellowish/reddish or violaceous papulo-vesicular lesions. CE usually shows blistering erythematous eruptions resembling erysipelas. However, CLC, TMBC, and CE are different clinical expressions of the same metastatic process, pathologically characterized by edema of the dermis and ectatic lymphatic vessels. Positivity to CD31 and podoplanin in the endothelial cells shows that the tumor metastatises predominantly via lymphatic vessels (20). In conclusion, we stress that every cutaneous lesion should be studied and examined carefully in patients with a personal history of cancer. Indeed, a correct diagnosis remains the pivotal point for a better management of these patients.

Renal cell carcinoma is an aggressive disease with a high rate of mortality. It is known to metastasize to the lung, liver, bone and brain. However, manifestation through lymphatic spread to the lungs is rare. Lymphangitic carcinomatosis is commonly observed in malignancies of the breast, lung, pancreas, colon and cervix. It is unusual to observe lymphangitic carcinomatosis of the lungs due to renal cell carcinoma. Lymphangitic carcinomatosis of the lungs may result in severe respiratory distress and may be the direct cause of death. Currently, there are no known modalities of preventing or slowing lymphangitic carcinomatosis besides treating the primary tumor. However, early detection may change the course of the disease and may prolong survival. This is compounded by the difficulty involved in diagnosing lymphangitic carcinomatosis of the lung which frequently involves lung biopsy. Immunohistochemical studies are often used in conjunction with regular histochemistry in ascertaining the primary tumor and in differentiating it from pulmonary metastasis. In this case report, we describe the presentation and clinical course of renal cell carcinoma in a patient which manifested as lymphangitis carcinomatosa of the lungs. The patient underwent surgical resection of the primary tumor with lymph node resection but presented with a fulminant lymphangitic carcinomatosis of the lungs within two weeks. Immunohistochemistry of the tissue obtained by the biopsy confirmed the diagnosis which was subsequently corroborated during his autopsy. This case illustrates the necessity of an urgent follow-up of chemotherapy and immunotherapy in such patients.

Ectopic adrenocorticotrophic hormone (ACTH) syndrome (EAS) commonly occurs secondary to neuroendocrine tumours and small cell carcinoma of lung. EAS has also been reported in association with gastric carcinoids. But, the occurrence of EAS secondary to gastric adenocarcinoma has rarely been reported. A 45-year-old male patient from Bangladesh presented with abdominal pain, jaundice and hyperpigmentation. Extensive work-up revealed poorly differentiating mucin-secreting adenocarcinoma of stomach with lymphangitis carcinomatosa of lung, bilateral adrenal metastasis and malignant common bile duct (CBD) stricture. Laboratory reports were suggestive for ectopic ACTH production. Most of these features are very rare in adenocarcinoma of stomach, and all these rare events occurring in a single patient is probably the rarest.

A 72-year-old female was admitted to the CCU with a recent onset of progressive breathlessness for bedside pericardial drainage for chronic pericardial effusion. After an uncomplicated drainage procedure, initially a serous straw coloured fluid was aspired with subsequent hemorrhagic aspiration with haemoglobin value similar to the peripheral blood. The patient showed initially transient improvement followed by rapid deterioration into severe shock and death. Signs of infero-posterior myocardial infarction (MI) were seen on the ECG. Before death, further interventions were refused by her and her family but a permission was given for autopsy. At autopsy, right ventricular rupture was seen with a 0.6 cm tear with a large amount of 800 cc bloody fluid with clots. The result of histopathologic study of the tear was resembling three-days old MI. The drain was found to be properly localized in the pericardial space, was not blocked and caused no harm to the myocardium. Furthermore, histopathologic examination revealed pulmonary adenocarcinoma of the left upper lobe, pleuritis and lymphangitis carcinomatosa and enlarged mediastinal lymph nodes. A case of fatal complication is reported following bedside pericardial drainage. Postmortal, right ventricular tear mimicked myocardial infarction.

Lymphoedema is a clinical condition caused by impairment of the lymphatic system, leading to swelling of subcutaneous soft tissues. As a result, accumulation of protein-rich interstitial fluid and lymphostasis often causes additional swelling, fibrosis and adipose tissue hypertrophy leading to progressive morbidity and loss of quality of life for the patient. Lymphoedema can be distinguished as primary or secondary. Lymphoedema is a complication frequently encountered in patients treated for cancer, especially after lymphadenoectomy and/or radiotherapy based on destruction of lymphatics. However, although lymphatic impairment is sometimes caused by obstructive solid metastasis, we present three cases of secondary lymphoedema with minor dermatological features without detectable solid metastasis. Sometimes this type of lymphoedema is mistakenly called malignant lymphoedema. All patients were previously treated for cancer without clinical signs of recurrence, presented with progressive lymphoedema and minor dermatological features of unknown origin. Clinical and histopathological examination of the skin revealed diffuse lymphangitis carcinomatosa, leading to secondary lymphoedema and adjustment of the therapeutic approach and prognosis. We reviewed literature on these rare presentations of cancer recurrence and recommend, where appropriate, consulting a dermatologist when discrete skin abnormalities are seen in patients with a history of cancer and developing lymphoedema.

Cutaneous lymphangitis carcinomatosa (CLC) is a rare form of cutaneous metastasis that causes lymphoedema and various eruptions. We report a case of lung cancer with CLC that caused both superior vena cava (SVC) stenosis and cervicofacial oedema, suggestive of SVC syndrome. A 64-year-old woman with lung adenocarcinoma presented with cervicofacial oedema and erythema, followed by severe dyspnoea 2 months after four cycles of carboplatin, pemetrexed and bevacizumab triplet therapy. Although chest CT indicated SVC stenosis, cervicofacial oedema remained despite treating the SVC stenosis via balloon dilation. A skin biopsy of the erythematic sample confirmed CLC as the cause of the patient's symptoms. CLC should be considered as a differential diagnosis of cervicofacial oedema in addition to SVC syndrome, especially when it is observed in combination with skin erythema and induration. Moreover, a skin biopsy should be performed promptly for accurate diagnosis of CLC and to decide on appropriate treatment.

An 83-year-old woman presented with erythema, papules and nodules on the left upper arm (figure 1A). She was treated with antibiotics by her general practitioner, with no disease response. Eight months earlier a 2.5 cm poorly differentiated primary cutaneous squamous cell carcinoma of the left preauricular area, with metastases to the regional lymph nodes, was staged T2N2M0 (tumour, node, metastases). She was treated with radical tumour resection, parotidectomy, and levels 2, 3 and 5 lymph node dissection, followed by postoperative radiotherapy. A total body skin examination was performed. On the left upper arm and adjacent to the axillary sulcus, a poor demarcated area of erythema with papules and nodules was noted. …

Lymphoedema is a clinical condition caused by impairment of the lymphatic system, leading to swelling of subcutaneous soft tissues. As a result, accumulation of protein-rich interstitial fluid and lymphostasis often causes additional swelling, fibrosis and adipose tissue hypertrophy leading to progressive morbidity and loss of quality of life for the patient. Lymphoedema can be distinguished as primary or secondary. Lymphoedema is a complication frequently encountered in patients treated for cancer, especially after lymphadenoectomy and/or radiotherapy based on destruction of lymphatics. However, although lymphatic impairment is sometimes caused by obstructive solid metastasis, we present three cases of secondary lymphoedema with minor dermatological features without detectable solid metastasis. Sometimes this type of lymphoedema is mistakenly called malignant lymphoedema. All patients were previously treated for cancer without clinical signs of recurrence, presented with progressive lymphoedema and minor dermatological features of unknown origin. Clinical and histopathological examination of the skin revealed diffuse lymphangitis carcinomatosa, leading to secondary lymphoedema and adjustment of the therapeutic approach and prognosis. We reviewed literature on these rare presentations of cancer recurrence and recommend, where appropriate, consulting a dermatologist when discrete skin abnormalities are seen in patients with a history of cancer and developing lymphoedema.

A clinicoradiological presentation of thoracic sarcoidosis requires histopathology in order to establish the diagnosis. Flexible bronchoscopy has a reasonable diagnostic yield and is the procedure of first choice for diagnosis. Endoscopic ultrasound (endoscopic ultrasound-guided fine needle aspiration/endobronchial ultrasound-guided transbronchial needle aspiration) can help in the diagnosis of sarcoidosis. An implementation strategy of endoscopic ultrasound for the diagnosis of sarcoidosis following negative flexible bronchoscopy results was examined prospectively in 15 clinics. A total of 137 patients (92 males; median age 43 yrs) were included, and sarcoidosis was found in 115 (84%). Alternative diagnoses were tuberculosis, lymphangitis carcinomatosa, pneumoconiosis and alveolitis. All patients were sent for flexible bronchoscopy, which was performed in 121 (88%), resulting in a definite diagnosis in 57 (42%). A total of 80 patients were sent for endoscopic ultrasound, which could be performed in 72 (90%), yielding a definite diagnosis in 47 (59%). Endoscopic ultrasound following negative flexible bronchoscopy avoided a surgical procedure in 47 out of 80 patients. The sensitivity of flexible bronchoscopy for sarcoidosis was 45% (95% confidence interval 3554%), but 62% (5072%) if biopsy specimens were taken. The sensitivity of endoscopic ultrasound following negative flexible bronchoscopy results was 71% (5882%). With this strategy, 97 out of 115 (84% (7690%)) of proven sarcoidosis was diagnosed using endoscopy. This large prospective implementation study (trial number NCT00888212 [ClinicalTrials.gov] ; ClinicalTrials.gov) shows that endoscopic ultrasound is valuable for diagnosing sarcoidosis after negative flexible bronchoscopy results.

Report on a patient in whom an extensively metastasizing medullary carcinoma of the thyroid was diagnosed at the age of 32 years. Noteworthy in her case history were watery diarrheas that had persisted for two years, suggesting that the medullary thyroid carcinoma had already existed for some time. Besides locoregional lymph node metastases, small foci of diffuse pulmonary metastases with lymphangitis carcinomatosa as well as osteoblastic skeletal metastases were present and intramammary metastases were detected later. The patient died from bilateral pleuropneumonia with empyema formation at the age of 47 years after an observation period of 15 years. During that period, no major changes in the pattern of metastatic spread were observed and one may therefore speak of "dormant" metastases in this case, although some manifestations showed increases and decreases in size.

Purpose To accurately define the gross tumor volume (GTV) and clinical target volume (GTV plus microscopic disease spread) for radiotherapy, the pretreatment imaging findings should be correlated with the histopathologic findings. In this pilot study, we investigated the feasibility of pathology-correlated imaging for lung tumors, taking into account lung deformations after surgery. Methods and Materials High-resolution multislice computed tomography (CT) and positron emission tomography (PET) scans were obtained for 5 patients who had non–small-cell lung cancer (NSCLC) before lobectomy. At the pathologic examination, the involved lung lobes were inflated with formalin, sectioned in parallel slices, and photographed, and microscopic sections were obtained. The GTVs were delineated for CT and autocontoured at the 42% PET level, and both were compared with the histopathologic volumes. The CT data were subsequently reformatted in the direction of the macroscopic sections, and the corresponding fiducial points in both images were compared. Hence, the lung deformations were determined to correct the distances of microscopic spread. Results In 4 of 5 patients, the GTV CT was, on average, 4 cm 3 (∼53%) too large. In contrast, for 1 patient (with lymphangitis carcinomatosa), the GTV CT was 16 cm 3 (∼40%) too small. The GTV PET was too small for the same patient. Regarding deformations, the volume of the well-inflated lung lobes on pathologic examination was still, on average, only 50% of the lobe volume on CT. Consequently, the observed average maximal distance of microscopic spread (5 mm) might, in vivo, be as large as 9 mm. Conclusions Our results have shown that pathology-correlated lung imaging is feasible and can be used to improve target definition. Ignoring deformations of the lung might result in underestimation of the microscopic spread.

Minute pulmonary meningothelial nodules are rare lesions histologically composed of small nests of epithelioid cells located within the interstitium of the lung. These nodules are generally asymptomatic and are usually found incidentally at autopsy or in surgical specimens resected for unrelated causes. The lesions are most often single, although multiple lesions with unilateral involvement of one or even all lobes of the same lung have been described. To our knowledge, cases of meningothelial nodules with disseminated bilateral pulmonary involvement associated with clinical symptoms of restrictive pulmonary disease and radiologic evidence of diffuse reticulonodular pulmonary infiltrates have not been previously documented. We have studied 5 patients presenting with diffuse bilateral pulmonary involvement by numerous minute pulmonary meningothelial nodules. The patients were 4 women and a man aged 54 to 75 years who presented clinically with dyspnea and shortness of breath and the lesions were discovered on open lung biopsies performed for the evaluation of diffuse bilateral interstitial lung infiltrates found on chest x-rays and computed tomography scans. In 3 patients, there was a previous history of malignancy and the radiologic findings were suspected of representing diffuse metastatic disease. Histologically, the lesions were composed of small clusters of epithelioid cells with round to oval nuclei devoid of atypia and surrounded by abundant eosinophilic cytoplasm. Immunohistochemical studies showed positivity of the tumor cells for epithelial membrane antigen and vimentin, and negative staining for cytokeratin, actin, S-100 protein, CD34, chromogranin, and synaptophysin. Electron microscopic examination in 1 case confirmed the ultrastructural features of meningothelial cells, including complex cytoplasmic interdigitations joined by well-developed desmosomes and abundant intracytoplasmic intermediate filaments. The diffuse bilateral involvement of lung parenchyma in the present cases can lead to confusion on clinical and radiologic grounds with a variety of interstitial pulmonary processes, including idiopathic interstitial pneumonia and lymphangitis carcinomatosa. Diffuse pulmonary meningotheliomatosis should be considered in the clinical differential diagnosis of diffuse interstitial pulmonary infiltrates.